Bmrb Entry 6269

BMRB Entry 6269

Title: 1H, 13C, and 15N Chemical Shift Assignments for chPrP(128-242) PubMed: 15459548

Authors: Lysek, Dominikus; Calzolai, Luigi; Wuthrich, Kurt

Citation: Lysek, Dominikus; Calzolai, Luigi; Wuthrich, Kurt. "Letters to the Editor: NMR assignment of the chicken prion protein fragments chPrP(128-242) and chPrP(25-242)" J. Biomol. NMR 30, 97-97 (2004).

Assembly members:chicken prion protein, polymer, 117 residues, Formula weight is not available

Natural source: Common Name: Chicken Taxonomy ID: 9031 Superkingdom: Eukaryota Kingdom: Metazoa Genus/species: Gallus gallus

Experimental source: Production method: recombinant technology

Assembly:

Entity 1, chPrP (128-242) 117 residues - Formula weight is not available

1 GLY SER VAL VAL GLY GLY LEU GLY GLY TYR

2 ALA MET GLY ARG VAL MET SER GLY MET ASN

3 TYR HIS PHE ASP ARG PRO ASP GLU TYR ARG

4 TRP TRP SER GLU ASN SER ALA ARG TYR PRO

5 ASN ARG VAL TYR TYR ARG ASP TYR SER SER

6 PRO VAL PRO GLN ASP VAL PHE VAL ALA ASP

7 CYS PHE ASN ILE THR VAL THR GLU TYR SER

8 ILE GLY PRO ALA ALA LYS LYS ASN THR SER

9 GLU ALA VAL ALA ALA ALA ASN GLN THR GLU

10 VAL GLU MET GLU ASN LYS VAL VAL THR LYS

11 VAL ILE ARG GLU MET CYS VAL GLN GLN TYR

12 ARG GLU TYR ARG LEU ALA SER

Samples:

sample_1: chicken prion protein, [U-13C; U-15N], 1.0 mM; sodium acetate 1.0 mM

Ex-cond_1: pH: 4.3; temperature: 293 K; ionic strength: 10 mM

Experiments:

Name SampleSample

stateSample

conditions

1H-15N NOESYnot

availablenot available not available

1H-13N NOESY aliphatic, aromatic

not available

not available not available

HNCAnot


HNCACBnot


METABOCARD

Cortexolone

Accession Number

HMDB00015

Created at 2005-11-16 15:48:42

Updated at 2012-01-10 17:19:04

Common Name

Cortexolone

Description

Cortexolone is the precursor of cortisol. Accumulation of Cortexolone can happen in a defect known as congenital adrenal hyperplasia, which is due to 11-beta-hydroxylase deficiency, resulting in androgen excess, virilization, and hypertension. (PMID: 2022736) A 17-hydroxycorticosteroid with glucocorticoid and anti-inflammatory activities. (PubChem)

Synonyms

1. 11-Deoxy-17-hydroxycorticosterone2. 11-Deoxycortisol3. 11-Deoxyhydrocortisone4. 11-Desoxy-17-hydroxycorticosterone5. 11-Desoxycortisol6. 11-Desoxyhydrocortisone7. 11-Dioxycortisol8. 11-deoxy-17-hydroxy-Corticosterone9. 11-deoxy-Cortisol10.11-dioxy-Cortisol11.17,21-Dihydroxy-4-pregnene-3,20-dione12.17,21-Dihydroxyprogesterone13.17,21-dihydroxypregn-4-ene-3,20-dione14.17-Hydroxy-11-deoxycorticosterone15.17alpha-Hydroxycortexone16.20-dione 17,21-Dihydroxypregn-4-ene-317.4-Pregnene-17alpha,21-diol-3,20-dione18.Cortodoxone19.Reichstein S20.Reichstein's compound S21.Reichstein's substance S

Chemical IUPAC Name

17-hydroxy-17-(2-hydroxyacetyl)-10,13-dimethyl-2,6,7,8,9,11,12,14,15,16-decahydro-1H-cyclopenta[a]phenanthren-3-one

Chemical C21H30O4

Formula

Chemical Structure

Chemical Taxonomy

Kingdom:Organic Compounds

Class:Steroids and Steroid Derivatives

Family:Not Available

Species:17-hydroxy-steroid20-keto-steroid3-keto-steroidcyclohexanecyclohexenecyclic alcoholtertiary alcoholalpha-ketoaldehydeketoneprimary alcoholdecaline

Biofunction:Hormones, Membrane component

Application:Not Available

Chemical Source

Endogenous

Molecular Weight

Monoisotopic: 346.21442 Average: 346.46100

Smiles (Isomeric)

C[C@]12CCC(=O)C=C1CC[C@@H]1C2CC[C@@]2(C)C1CC[C@]2(O)C(=O)CO

Smiles (Canonical)

CC12CCC(=O)C=C1CCC1C2CCC2(C)C1CCC2(O)C(=O)CO

KEGG Compound ID

C05488

BioCyc ID 11-DEOXY-CORTISOL

BiGG ID 45914

Wikipedia ID Cortexolone

METLIN ID 5088

PubChem Compound ID

53477676

PubChem Substance ID(s)

3135470 ; 7978569 ; 7847 ; 855521 ; 10298640 ; 841445

ChEBI ID Not Available

CAS Registry Number

152-58-9

InChI Identifier

InChI=1/C21H30O4/c1-19-8-5-14(23)11-13(19)3-4-15-16(19)6-9-20(2)17(15)7-10-21(20,25)18(24)12-22/h11,15-17,22,25H,3-10,12H2,1-2H3/t15-,16+,17?,19+,20+,21+/m1/s1

Synthesis Reference

Luu, Duc Huy. New method for the synthesis of Reichstein's compound S from pregnene. Tap Chi Duoc Hoc (2002), (9), 23-25.

Melting Point (Experimental)

215 C

H2O Solubility (Experimental)

Not Available

H2O Solubility (Predicted)

0.0152 mg/mL [MEYLAN,WM et al. (1996)]0.025 mg/mL [Predicted by ALOGPS]

StateSolid

LogP/Hydrophobicity (Experimental)

3.08 [SANGSTER (1994)]

LogP/Hydrophobicity (Predicted)

2.79 [Predicted by ALOGPS]; 2.4 [Predicted by PubChem via XLOGP]

Material Safety Data Sheet (MSDS)

Download File

MOL File (Image)

MOL File (Text)

Download File

SDF File (Text)

Download File

PDB File Calculated (Image)

PDB File Calculated (Text)

Download File

PDB ID Not Available

PDB File Experimental (Text)

Not Available

PDB File Experimental (Image)

Not Available

Experimental 1H NMR Spectrum

Download SpectrumView Experimental ConditionsDownload FID (Varian)

Experimental 13C NMR Spectrum

Not Available

Experimental 13C HSQC Spectrum

Download SpectrumView Experimental ConditionsDownload FID (Bruker)

Predicted 1H NMR Spectrum

Download File

Predicted 13C NMR

Download File

View 2D Structure

View 3D Structure

Spectrum

Mass Spectrum

Download File (Low Energy) View Experimental ConditionsDownload File (Medium Energy) View Experimental ConditionsDownload File (High Energy) View Experimental Conditions

Simplied Tocsy Spectrum

Not Available

BMRB Spectrum

Not Available

Cellular Location

1. Membrane (Predicted from LogP)2. Cytoplasm (Predicted from Solubility)3. Cytoplasm4. endoplasmic reticulum5. mitochondria6. Extracellular

Biofluid Location

1. Blood2. Urine

Tissue Location

All Tissues

Concentration (Normal)

Biofluid Blood

Value 0.0039 +/- 0.0004 uM

Age Adult:>18 yrs old

Sex Both

Condition Normal

References

Tjeerdsma G, Sluiter WJ, Hew JM, Molenaar WM, de Lange WE, Dullaart RP: Hyperprolactinaemia is associated with a higher prevalence of pituitary-adrenal dysfunction in non-functioning pituitary macroadenoma. Eur J Endocrinol. 1996 Sep;135(3):299-308. [PubMed]

Biofluid Urine

Value 0.017-178.9 umol/mmol creatinine

Age Newborn:0-30 days old

Sex Both

Condition Normal

References

Hughes IA, Arisaka O, Perry LA, Honour JW: Early diagnosis of 11 beta-hydroxylase deficiency in two siblings confirmed by analysis of a novel steroid metabolite in newborn urine. Acta Endocrinol (Copenh). 1986 Mar;111(3):349-54. [PubMed]

Biofluid Urine

Value 0.02 (0.0091-0.032) umol/mmol creatinine


Sex Female

Condition Normal

ReferencesZachmann M, Tassinari D, Prader A: Clinical and biochemical variability of congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. A study of 25 patients. J Clin Endocrinol Metab. 1983 Feb;56(2):222-9. [

Concentration (Abnormal)

Not Available

Associated Disorders

1. 11-@beta-hydroxylase deficiency2. 18-@hydroxysteroid dehydrogenase deficiency, included3. 21-@hydroxylase deficiency4. Adrenal hyperplasia III5. Adrenal hyperplasia iv6. Adrenal hyperplasia, congenital, due to 11-beta-hydroxylase

deficiency7. Adrenal hyperplasia, congenital, due to 21-hydroxylase

deficiency8. CYP21 deficiency9. Congenital adrenal hyperplasia 110.Hyperandrogenism, nonclassic type, due to 21-hydroxylase

deficiency,11.Hypertensive form of adrenal hyperplasia12.P450C11B1 deficiency

OMIM ID mim:201910 (Adrenal hyperplasia, congenital, due to 21-hydroxylase deficiency; Adrenal hyperplasia III; 21-@hydroxylase deficiency; CYP21 deficiency; Congenital adrenal hyperplasia 1; CAH1; Cytochrome P450, subfamily xxia, polypeptide 2, included; Cyp21a2,; Included; Cytochrome P450, subfamily xxi, included; CYP21, included; Steroid

cytochrome P450 21-hydroxylase, included; P450C21, included; 21-@hydroxylase b, included; CYP21B, included; Ca21h, included; Cytochrome P450, subfamily xxia, polypeptide 1 pseudogene, included; Cyp21a1p, included; CYP21P, included; Cyp21a, included; Hyperandrogenism, nonclassic type, due to 21-hydroxylase deficiency,)mim:202010 (Adrenal hyperplasia, congenital, due to 11-beta-hydroxylase deficiency; Adrenal hyperplasia iv; 11-@beta-hydroxylase deficiency; Hypertensive form of adrenal hyperplasia; Steroid 11-beta-hydroxylase; P450C11B1 deficiency; Cytochrome P450, subfamily xib, polypeptide 1, included; CYP11B1,; Included; 18-@hydroxysteroid dehydrogenase deficiency, included)

Metagene ID Not Available

Pathway Names

Not Available

KEGG Images

Not Available

SimCell Pathway Images

Not Available

SimCell Pathway Graphs

Not Available

SimCell Pathway SBMLs (View)

Not Available

SimCell Pathway SBMLs (Download)

Not Available

General References

1. Homma K, Hasegawa T, Masumoto M, Takeshita E, Watanabe K, Chiba H, Kurosawa T, Takahashi T, Matsuo N: Reference values for urinary steroids in Japanese newborn infants: gas chromatography/mass spectrometry in selected ion monitoring. Endocr J. 2003 Dec;50(6):783-92. [PubMed]

2. Trakakis E, Laggas D, Salamalekis E, Creatsas G: 21-Hydroxylase deficiency: from molecular genetics to clinical presentation. J Endocrinol Invest. 2005 Feb;28(2):187-92. [PubMed]

3. Krone N, Riepe FG, Gotze D, Korsch E, Rister M, Commentz J,

Partsch CJ, Grotzinger J, Peter M, Sippell WG: Congenital adrenal hyperplasia due to 11-hydroxylase deficiency: functional characterization of two novel point mutations and a three-base pair deletion in the CYP11B1 gene. J Clin Endocrinol Metab. 2005 Jun;90(6):3724-30. Epub 2005 Mar 8. [PubMed]

4. Adinoff B, Krebaum SR, Chandler PA, Ye W, Brown MB, Williams MJ: Dissection of hypothalamic-pituitary-adrenal axis pathology in 1-month-abstinent alcohol-dependent men, part 1: adrenocortical and pituitary glucocorticoid responsiveness. Alcohol Clin Exp Res. 2005 Apr;29(4):517-27. [PubMed]

5. Brown JR, Cavanaugh AH, Farnsworth WE: A simple radioimmunoassay for plasma cortisol and 11-deoxycortisol (17, 21-dihydroxy-4-pregnene-3, 20-dione). Steroids. 1976 Oct;28(4):487-98. [PubMed]

6. den Brinker M, Joosten KF, Liem O, de Jong FH, Hop WC, Hazelzet JA, van Dijk M, Hokken-Koelega AC: Adrenal insufficiency in meningococcal sepsis: bioavailable cortisol levels and impact of interleukin-6 levels and intubation with etomidate on adrenal function and mortality. J Clin Endocrinol Metab. 2005 Sep;90(9):5110-7. Epub 2005 Jun 28. [PubMed]

7. Lee HH, Won GS, Chao HT, Lee YJ, Chung BC: Novel missense mutations, GCC [Ala306]- > GTC [Val] and ACG [Thr318]- > CCG [Pro], in the CYP11B1 gene cause steroid 11beta-hydroxylase deficiency in the Chinese. Clin Endocrinol (Oxf). 2005 Apr;62(4):418-22. [PubMed]

8. Zachmann M, Tassinari D, Prader A: Clinical and biochemical variability of congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. A study of 25 patients. J Clin Endocrinol Metab. 1983 Feb;56(2):222-9. [PubMed]

9. White PC, Tusie-Luna MT, New MI, Speiser PW: Mutations in steroid 21-hydroxylase (CYP21). Hum Mutat. 1994;3(4):373-8. [PubMed]

10.Bradshaw KD, Milewich L, Mason JI, Parker CR Jr, MacDonald PC, Carr BR: Steroid secretory characteristics of a virilizing adrenal adenoma in a woman. J Endocrinol. 1994 Feb;140(2):297-307. [PubMed]

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12.Dragan CA, Zearo S, Hannemann F, Bernhardt R, Bureik M: Efficient conversion of 11-deoxycortisol to cortisol (hydrocortisone) by recombinant fission yeast Schizosaccharomyces pombe. FEMS Yeast Res. 2005 Apr;5(6-7):621-5. [PubMed]

13.Mellon SH, Miller WL: Extraadrenal steroid 21-hydroxylation is

not mediated by P450c21. J Clin Invest. 1989 Nov;84(5):1497-502. [PubMed]

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15.Tjeerdsma G, Sluiter WJ, Hew JM, Molenaar WM, de Lange WE, Dullaart RP: Hyperprolactinaemia is associated with a higher prevalence of pituitary-adrenal dysfunction in non-functioning pituitary macroadenoma. Eur J Endocrinol. 1996 Sep;135(3):299-308. [PubMed]

16.Hughes IA, Arisaka O, Perry LA, Honour JW: Early diagnosis of 11 beta-hydroxylase deficiency in two siblings confirmed by analysis of a novel steroid metabolite in newborn urine. Acta Endocrinol (Copenh). 1986 Mar;111(3):349-54. [PubMed]

17.Curnow KM, Tusie-Luna MT, Pascoe L, Natarajan R, Gu JL, Nadler JL, White PC: The product of the CYP11B2 gene is required for aldosterone biosynthesis in the human adrenal cortex. Mol Endocrinol. 1991 Oct;5(10):1513-22. [PubMed]

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21.Zieger B, Hubl W, Buchner M: [Interpretation of the metopirone--midnight-short-test by means of simultaneous determination and evaluation of the increase of 11-deoxycortisol and decrease of plasma 11-hydroxycorticosteroids] Z Gesamte Inn Med. 1975 Aug 15;30(16):539-45. [PubMed]

22.Zhu YS, Cordero JJ, Can S, Cai LQ, You X, Herrera C, DeFillo-Ricart M, Shackleton C, Imperato-McGinley J: Mutations in CYP11B1 gene: phenotype-genotype correlations. Am J Med Genet A. 2003 Oct 15;122(3):193-200. [PubMed]

23.Boyar RM, Nogeire C, Fukushima D, Hellman L, Fishman J: Studies of the diurnal pattern of plasma corticosteroids and gonadotropins in two cases of feminizing adrenal carcinoma: measurements of estrogen and corticosteroid production. J Clin Endocrinol Metab. 1977 Jan;44(1):39-45. [PubMed]

24.Keavney B, Mayosi B, Gaukrodger N, Imrie H, Baker M, Fraser R, Ingram M, Watkins H, Farrall M, Davies E, Connell J: Genetic variation at the locus encompassing 11-beta hydroxylase and aldosterone synthase accounts for heritability in cortisol

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25.Verhelst JA, Trainer PJ, Howlett TA, Perry L, Rees LH, Grossman AB, Wass JA, Besser GM: Short and long-term responses to metyrapone in the medical management of 91 patients with Cushing's syndrome. Clin Endocrinol (Oxf). 1991 Aug;35(2):169-78. [PubMed]

26.Plonk J, Feldman J: Modification of adrenal function by the anti-serotonin agent cyproheptadine. J Clin Endocrinol Metab. 1976 Feb;42(2):291-5. [PubMed]

27.Wand GS, Dobs AS: Alterations in the hypothalamic-pituitary-adrenal axis in actively drinking alcoholics. J Clin Endocrinol Metab. 1991 Jun;72(6):1290-5. [PubMed]

28.Dolman LI, Nolan G, Jubiz W: Metyrapone test with adrenocorticotrophic levels. Separating primary from secondary adrenal insufficiency. JAMA. 1979 Mar 23;241(12):1251-3. [PubMed]

29.Underwood RH, Bradwin GR, Moore TJ, Williams GH: Semi-automated high-performance liquid chromatographic method for the simultaneous assay of plasma cortisol and 11-deoxycortisol in the metyrapone test. J Chromatogr. 1990 Mar 16;526(1):180-5. [PubMed]

30.Govaerts L, Sippell WG, Monnens L: Further analysis of the disturbed adrenocortical function in the cerebro-hepato-renal syndrome of Zellweger. J Inherit Metab Dis. 1989;12(4):423-8. [PubMed]

31.Konta A, Osanai T, Umemura Y, Matsumura H, Kikuchi T, Eidoh H, Akiba R, Kanazawa T, Onodera K, Sobajima T, et al.: [A case of the benign adrenal adenoma with remarkably high concentrations of deoxycorticosterone and 11-deoxycortisol] Nippon Jinzo Gakkai Shi. 1989 Nov;31(11):1223-7. [PubMed]

32.Leal-Cerro A, Garcia-Luna PP, Villar J, Miranda ML, Pereira JL, Gomez-Pan A, Astorga R: Arterial hypertension as a complication of prolonged ketoconazole treatment. J Hypertens Suppl. 1989 Dec;7(6):S212-3. [PubMed]

33.Dorr HG, Heller A, Versmold HT, Sippell WG, Herrmann M, Bidlingmaier F, Knorr D: Longitudinal study of progestins, mineralocorticoids, and glucocorticoids throughout human pregnancy. J Clin Endocrinol Metab. 1989 May;68(5):863-8. [PubMed]

34.Bezverkhaia TP, Rybakov OI, Komissarenko IV, Verkhogliadova LM: [Glucocorticoids in patients with tumors of the adrenal cortex] Probl Endokrinol (Mosk). 1989 Jan-Feb;35(1):11-5. [PubMed]

35.Hurwitz A, Brautbar C, Milwidsky A, Vecsei P, Milewicz A, Navot

D, Rosler A: Combined 21- and 11 beta-hydroxylase deficiency in familial congenital adrenal hyperplasia. J Clin Endocrinol Metab. 1985 Apr;60(4):631-8. [PubMed]

36.Yamakita N, Yasuda K, Murase H, Murayama M, Goshima E, Hirai M, Noritake N, Takeda N, Miura K: Response of plasma adrenal steroids to synthetic ACTH under ketoconazole in man. Endocrinol Jpn. 1987 Feb;34(1):29-35. [PubMed]

37.Rodriguez Portales JA, Arteaga E, Lopez Moreno JM, Biglieri EG: Zona glomerulosa function after life-long suppression in two siblings with the hypertensive virilizing form of congenital adrenal hyperplasia. J Clin Endocrinol Metab. 1988 Feb;66(2):349-54. [PubMed]

38.Lewis JG, Yeo KH, Elder PA: A competitive enzyme-linked immunosorbent assay for plasma 11-deoxycortisol (17,21-dihydroxy-4-pregnene-3,20-dione). Steroids. 1986 Jun;47(6):365-72. [PubMed]

39.Dickstein G, Lahav M, Orr ZS: Single-dose metyrapone test at 06.00 h: an accurate method for assessment of pituitary-adrenal reserve. Acta Endocrinol (Copenh). 1986 May;112(1):28-34. [PubMed]

40.Cawson MJ, Anderson AB, Turnbull AC, Lampe L: Cortisol, cortisone, and 11-deoxycortisol levels in human umbilical and maternal plasma in relation to the onset of labour. J Obstet Gynaecol Br Commonw. 1974 Oct;81(10):737-45. [PubMed]

41.Fukushima DK, Nishina T, Wu RH, Hellman L, Finkelstein JW: Rapid assay of plasma 21-deoxycortisol and 11-deoxycortisol in congenital adrenal hyperplasia. Clin Endocrinol (Oxf). 1979 Apr;10(4):367-75. [PubMed]

42.Hubl W, Stahl F, Freymann E, Buchner M: [A simple method for the determination of 11-desoxycortisol in small amounts of plasma using competitive protein binding analysis] Clin Chim Acta. 1973 Dec 27;49(3):423-33. [PubMed]

43.Newsome HH Jr, Clements AS, Borum EH: The simultaneous assay of cortisol, corticosterone, 11-deoxycortisol, and cortisone in human plasma. J Clin Endocrinol Metab. 1972 Mar;34(3):473-83. [PubMed]

44.Maschler I, Weidenfeld J, Muller A, Slavin S, Shaefer J, Chowers I, Finkelstein M: A case of adrenogenital syndrome with aberrant 11beta-hydroxylation. Acta Endocrinol (Copenh). 1977 Aug;85(4):832-9. [PubMed]

45.Dickstein G, Barzilai D: Improved single-dose metyrapone test. Isr J Med Sci. 1980 May;16(5):365-9. [PubMed]

46.Castro-Magana M, Cheruvanky T, Collipp PJ, Ghavami-Maibodi Z, Angulo M, Stewart C: Transient adrenogenital syndrome due to exposure to danazol in utero. Am J Dis Child. 1981 Nov;135(11):1032-4. [PubMed]

47.Whitworth JA, Saines D, Thatcher R, Butkus A, Scoggins BA, Coghlan JP: Blood pressure, renal and metabolic effects of ACTH in normotensive man. Clin Sci (Lond). 1981 Dec;61 Suppl 7:269s-272s. [PubMed]

48.Glickman SP, Rosenfield RL, Bergenstal RM, Helke J: Multiple androgenic abnormalities, including elevated free testosterone, in hyperprolactinemic women. J Clin Endocrinol Metab. 1982 Aug;55(2):251-7. [PubMed]

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62.Peter M, Sippell WG: Evidence for endocrinological abnormalities in heterozygotes for adrenal 11 beta-hydroxylase deficiency of a family with the R448H mutation in the CYP11B1 gene. J Clin Endocrinol Metab. 1997 Oct;82(10):3506-8. [PubMed]

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Metabolic Enzymes

Not Available

METABOCARD

Carnosine

Accession Number

HMDB00033

Created at

2005-11-16 15:48:42

Updated at

2009-06-30 15:15:26

Common Name

Carnosine

Description

Carnosine (beta-alanyl-L-histidine) is found exclusively in animal tissues. It is a dipeptide of the amino acids beta-alanine and histidine. Carnosine has the potential to suppress many of the biochemical changes (e.g., protein oxidation, glycation, AGE formation, and cross-linking) that accompany aging and associated pathologies (PMID 16804013). It is highly concentrated in muscle and brain tissues. Some autistics patients take it as a dietary supplement, and attribute an improvement in their condition to it. Supplemental carnosine may increase corticosterone levels. This may explain the "hyperactivity" seen in autistic subjects at higher doses. Carnosine also exhibits some antioxidant effects. The antioxidant mechanism of carnosine is attributed to its chelating effect against metal ions, superoxide dismutase (SOD)-like activity, ROS and free radicals scavenging ability (PMID 16406688)

Synonyms

1. Beta-Alanyl-L-histidine2. Carnosine3. Ignotine4. Karnozin5. Karnozzn6. L-Carnosine7. N-(3-Aminopropanoyl)histidine8. N-(b-Alanyl)-L-histidine9. N-b-alanyl-L-Histidine10.N-beta-alanyl-L-Histidine11.b-Alanyl-L-histidine12.b-Alanylhistidine13.beta-Alanylhistidine

14.Sevitin

Chemical IUPAC Name

(2S)-2-(3-aminopropanoylamino)-3-(3H-imidazol-4-yl)propanoic acid

Chemical Formula

C9H14N4O3

Chemical Structure

Chemical Taxonomy

Kingdom:Organic Compounds

Class:Peptidomimetics

Family:Not Available

Species:n-acyl-alpha-amino-acidbeta amino acid or derivativen-substituted-alpha-amino acidimidazolyl carboxylic acid derivativeimidazolesecondary carboxylic acid amidecarboxamide groupcarboxylic acidprimary aliphatic amine (alkylamine)

Biofunction:Component of beta-Alanine metabolism; Component of Histidine metabolism

Application:Not Available

Chemical Source

Endogenous

Molecular Weight

Monoisotopic: 226.10658 Average: 226.23200

Smiles (Isomeric)

NCCC(=O)N[C@@H](CC1=CN=CN1)C(O)=O

Smiles (Canonical)

NCCC(=O)NC(CC1=CN=CN1)C(O)=O

KEGG Compound ID

C00386

BioCyc ID

CARNOSINE

BiGG ID

1800369

Wikipedia ID

Carnosine

METLIN ID

38

PubChem Compound ID

439224

PubChem Substance ID(s)

11366634 ; 3676 ; 11494992 ; 11488270 ; 10298255 ; 11537748 ; 11377358 ; 11491027 ; 11369196 ; 11361828 ; 11113307 ; 8143363 ; 11487230 ;3138777 ; 11364072 ; 11493487 ; 11484264 ; 11375363 ; 11341645 ; 11372223

ChEBI ID

15727

CAS Registry Number

305-84-0

InChI Identifier

InChI=1/C9H14N4O3/c10-2-1-8(14)13-7(9(15)16)3-6-4-11-5-12-6/h4-5,7H,1-3,10H2,(H,11,12)(H,13,14)(H,15,16)/t7-/m0/s1

Synthesis Referen

Vezenkov, L.; Yanachkov, O. Synthesis of carnosine by trimethylsilyl protection. Dokladi na Bulgarskata Akademiya na Naukite (1991), 44(8), 53-6

ce

Melting Point (Experimental)

253 - 256 C

H2O Solubility (Experimental)

384 mg/mL [HMP experimental]

H2O Solubility (Predicted)

11.1 mg/mL [Predicted by ALOGPS]82.1 mg/mL at 25 °C [MEYLAN,WM et al. (1996)]

StateSolid

LogP/Hydrophobicity (Experimental)

Not Available

LogP/Hydrophobicity (Predicted)

-2.96 [Predicted by ALOGPS]; -4.1 [Predicted by PubChem via XLOGP]; -1.81 [MEYLAN,WM & HOWARD,PH (1995)]

Material Safety Data Sheet (MSDS)

Download File

MOL File (Image)

MOL File (Text)

Download File

View 2D Structure

SDF File (Text)

Download File

PDB File Calculated (Image)

PDB File Calculated (Text)

Download File

PDB ID Not Available

PDB File Experimental (Text)

Not Available

PDB File Experimental (Image)

Not Available

Experimental 1H NMR Spectrum

Download SpectrumView Experimental ConditionsDownload FID (Varian)

Experimental 13C NMR Spectrum


Experimental 13C HSQC Spectrum


View 3D Structure

Predicted 1H NMR Spectrum

Download File

Predicted 13C NMR Spectrum

Download File

Mass Spectrum

Download File (Low Energy) View Experimental ConditionsDownload File (Medium Energy) View Experimental ConditionsDownload File (High Energy) View Experimental Conditions

Simplied Tocsy Spectrum

Download File View Peaklist

BMRB Spectrum

Download File View Peaklist

Cellular Location

Cytoplasm

Biofluid Location

1. Blood2. CSF3. Urine

Tissue Location

1. Brain2. Fibroblasts3. Intestine4. Kidney5. Liver6. Muscle7. Neurons8. Skeletal Muscle

Concentration (Normal)

Biofluid Blood

Value 6.54 +/- 1.0 uM


Sex Both

Condition Normal

ReferencesFonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [

Biofluid Blood

Value 3.1 +/- 7.5 uM

Age Infant:0-1 yr old

Sex Both

Condition Normal

ReferencesGeigy Scientific Tables, 8th Rev edition, pp. 80-97. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.

Biofluid Urine

Value 1.84 umol/mmol creatinine


Sex Both

Condition Normal

ReferencesGuo K, Li L: Differential (12)C-/(13)C-Isotope Dansylation Labeling and Fast Liquid Chromatography/Mass Spectrometry for Absolute and Relative Quantification of the Metabolome. Anal Chem. 2009 Mar 23. [

Biofluid Urine

Value 19.97 umol/mmol creatinine


Sex Male

Condition Normal

ReferencesShaykhutdinov RA, MacInnis GD, Dowlatabadi R, Weljie AM, Vogel HJ. Quantitative analysis of metabolite concentrations in human urine samples using 13C{1H} NMR spectroscopy. Metabolomics. 2009

Biofluid Urine

Value 3.2 +/- 4.36 umol/mmol creatinine

Age Children:1-13 yrs old

Sex Female

Condition Normal

References

Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by Cornelius Lentner.West Cadwell, N.J. : Medical education Div., Ciba-Geigy Corp.Basel, Switzerland c1981-1992.

Biofluid Urine



Sex Male

Condition Normal

References


Biofluid Urine



Sex Female

Condition Normal

References


Biofluid Urine



Sex Not Available

Condition Normal

ReferencesDoctor's Data

Biofluid Urine

Value 1.1+/-0.5 umol/mmol creatinine

Age Not Available

Sex Both

Condition Normal

ReferencesWilli SM, Zhang Y, Hill JB, Phelan MC, Michaelis RC, Holden KR: A deletion in the long arm of chromosome 18 in a child with serum carnosinase deficiency. Pediatr Res. 1997 Feb;41(2):210-3. [

Biofluid Urine



Sex Both

Condition Normal

ReferencesBouatra, S. et al. (2012) The Human Urine Metabolome (manuscript in preparation)

Concentration (Abnormal)

Biofluid Blood

Value 3.28 +/- 0.91 uM

Age Elderly:>65 yrs old

Sex Both

Condition Alzheimer's disease


Biofluid Cerebrospinal Fluid(CSF)

Value <0.1 uM

Age Not Available

Sex Not Available

Condition Not Available

References

Peters V, Kebbewar M, Jansen EW, Jakobs C, Riedl E, Koeppel H, Frey D, Adelmann K, Klingbeil K, Mack M, Hoffmann GF, Janssen B, Zschocke J, Yard BA: Relevance of allosteric conformations and homocarnosine concentration on carnosinase activity. Amino Acids. 2010 May;38(5):1607-15. Epub 2009 Nov 14. [

Biofluid Urine



Sex Both

Condition Normal

Referenceshttp://www.metagene.de/program/d.prg?mp=CARNOSINURIA

Biofluid Urine



Sex Both

Condition Carnosinuria

Referenceshttp://www.metagene.de/program/d.prg?mp=CARNOSINURIA

Biofluid Urine



Sex Both

Condition Alzheimer's disease


Biofluid Urine



Sex Not Available

Condition Carnosinuria

ReferencesFurst P: Amino acid metabolism in uremia. J Am Coll Nutr. 1989 Aug;8(4):310-23. [

Associated Disorders

1. Alzheimer's disease2. Carnosinase deficiency3. Carnosinemia4. Carnosinuria5. Gaba transaminase deficiency6. Homocarnosinase deficiency7. Hyperalaninemia8. Normal

OMIM ID

mim:212200 (Carnosinemia; Carnosinase deficiency)mim:236130 (Homocarnosinase deficiency)mim:237400 (Hyperalaninemia)

Metagene ID

32 (Gaba transaminase deficiency)82 (Carnosinuria)

Pathway Names

Not Available

KEGG Not Available

Images

SimCell Pathway Images

Not Available

SimCell Pathway Graphs

Not Available

SimCell Pathway SBMLs (View)

Not Available

SimCell Pathway SBMLs (Download)

Not Available

General References

1. Park YJ, Volpe SL, Decker EA: Quantitation of carnosine in humans plasma after dietary consumption of beef. J Agric Food Chem. 2005 Jun 15;53(12):4736-9. [PubMed]

2. Guiotto A, Calderan A, Ruzza P, Borin G: Carnosine and carnosine-related antioxidants: a review. Curr Med Chem. 2005;12(20):2293-315. [PubMed]

3. Hipkiss AR, Preston JE, Himsworth DT, Worthington VC, Keown M, Michaelis J, Lawrence J, Mateen A, Allende L, Eagles PA, Abbott NJ: Pluripotent protective effects of carnosine, a naturally occurring dipeptide. Ann N Y Acad Sci. 1998 Nov 20;854:37-53. [PubMed]

4. Guiotto A, Calderan A, Ruzza P, Osler A, Rubini C, Jo DG, Mattson MP, Borin G: Synthesis and evaluation of neuroprotective alpha,beta-unsaturated aldehyde scavenger histidyl-containing analogues of carnosine. J Med Chem. 2005 Sep 22;48(19):6156-61. [PubMed]

5. Hipkiss AR, Brownson C: Carnosine reacts with protein carbonyl groups: another possible role for the anti-ageing peptide? Biogerontology. 2000;1(3):217-23. [PubMed]

6. Furst P: Amino acid metabolism in uremia. J Am Coll Nutr. 1989 Aug;8(4):310-23. [PubMed]

7. Lee YT, Hsu CC, Lin MH, Liu KS, Yin MC: Histidine and carnosine delay diabetic deterioration in mice and protect human low density lipoprotein against oxidation and glycation. Eur J Pharmacol. 2005 Apr 18;513(1-2):145-50. Epub 2005 Apr 2. [PubMed]

8. Hipkiss AR, Brownson C, Bertani MF, Ruiz E, Ferro A: Reaction of carnosine with aged proteins: another protective process? Ann N Y Acad Sci. 2002 Apr;959:285-94. [PubMed]

9. Mannion AF, Jakeman PM, Dunnett M, Harris RC, Willan PL: Carnosine and anserine concentrations in the quadriceps femoris muscle of healthy humans. Eur J Appl Physiol Occup Physiol. 1992;64(1):47-50. [PubMed]

10.Hipkiss AR, Brownson C, Carrier MJ: Carnosine, the anti-ageing, anti-oxidant dipeptide, may react with protein carbonyl groups. Mech Ageing Dev. 2001 Sep 15;122(13):1431-45. [PubMed]

11.Suzuki Y, Ito O, Mukai N, Takahashi H, Takamatsu K: High level of skeletal muscle carnosine contributes to the latter half of exercise performance during 30-s maximal cycle ergometer sprinting. Jpn J Physiol. 2002 Apr;52(2):199-205. [PubMed]

12.Kang JH, Kim KS, Choi SY, Kwon HY, Won MH, Kang TC: Protection by carnosine-related dipeptides against hydrogen peroxide-mediated ceruloplasmin modification. Mol Cells. 2002 Feb 28;13(1):107-12. [PubMed]

13.Willi SM, Zhang Y, Hill JB, Phelan MC, Michaelis RC, Holden KR: A deletion in the long arm of chromosome 18 in a child with serum carnosinase deficiency. Pediatr Res. 1997 Feb;41(2):210-3. [PubMed]

14.Tallon MJ, Harris RC, Boobis LH, Fallowfield JL, Wise JA: The carnosine content of vastus lateralis is elevated in resistance-trained bodybuilders. J Strength Cond Res. 2005 Nov;19(4):725-9. [PubMed]

15.Mozdzan M, Szemraj J, Rysz J, Nowak D: Antioxidant properties of carnosine re-evaluated with oxidizing systems involving iron and copper ions. Basic Clin Pharmacol Toxicol. 2005 May;96(5):352-60. [PubMed]

16.Jackson MC, Lenney JF: The distribution of carnosine and related dipeptides in rat and human tissues. Inflamm Res. 1996 Mar;45(3):132-5. [PubMed]

17.Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed]

18.Geigy Scientific Tables, 8th Rev edition, pp. 80-97. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.

19.Peters V, Kebbewar M, Jansen EW, Jakobs C, Riedl E, Koeppel H, Frey D, Adelmann K, Klingbeil K, Mack M, Hoffmann GF, Janssen B, Zschocke J, Yard BA: Relevance of allosteric conformations and homocarnosine concentration on carnosinase activity. Amino Acids. 2010 May;38(5):1607-15. Epub 2009 Nov 14. [PubMed]

20.Guo K, Li L: Differential (12)C-/(13)C-Isotope Dansylation Labeling and Fast Liquid Chromatography/Mass Spectrometry for Absolute and Relative Quantification of the Metabolome. Anal Chem. 2009 Mar 23.

[PubMed]21.http://www.metagene.de/program/d.prg?mp=CARNOSINURIA22.http://www.metagene.de/program/d.prg?mp=CARNOSINURIA23.Shaykhutdinov RA, MacInnis GD, Dowlatabadi R, Weljie AM, Vogel

HJ. Quantitative analysis of metabolite concentrations in human urine samples using 13C{1H} NMR spectroscopy. Metabolomics. 2009

24.Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by Cornelius Lentner.

25.West Cadwell, N.J. : Medical education Div., Ciba-Geigy Corp.26.Basel, Switzerland c1981-1992.27.Cheng Y, Xie G, Chen T, Qiu Y, Zou X, Zheng M, Tan B, Feng B,

Dong T, He P, Zhao L, Zhao A, Xu LX, Zhang Y, Jia W: Distinct urinary metabolic profile of human colorectal cancer. J Proteome Res. 2012 Feb 3;11(2):1354-63. Epub 2011 Dec 28. [PubMed]

28.Doctor's Data29.Bouatra, S. et al. \(2012\) The Human Urine Metabolome (manuscript

in preparation)30.Pubmed: 1680401331.Pubmed: 16406688

Metabolic Enzymes

Not Available

Bmrb Entry 6269

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