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International Journal of Current Research inSciences

ISSN: 2454-5716www.ijcrims.com

Coden: IJCRPP(USA)

Review Articlehttp://s-o-i.org/1.15/ijcrms-2016-2-1-9

MEDICAL ASPECTS OF CARBOHYDRATES:HETEROPOLYSACCHARIDES

Dr. Anil Batta

Associate Professor Department of Biochemistry G. G. S. Medical College and Hospital Faridkot*Corresponding author: akbattafarid@yahoo.co.in

Abstract

GAGs are long, negatively charged, unbranched heteropolysacch chains generally composed of a repeatingdisaccharide unit [acidic sugar-amino sugar]n • The amino sugar is either D-glucosamine or D- galactosamine inwhich the amino group is usually acetylated, thus eliminating its +ve charge.The amino sugar may also be sulfated onC-4 or 6 or on a non-acetylated nitrogen. The acidic sugar is either D-glucuronic acid or its C- 5 epimer, L-iduronicacid. They bind large amounts of water, thereby producing the gel-like matrix that forms the basis of the body’sground substance. • The viscous, lubricating properties of mucous secretions are also caused by the presence of GAGswhich led to the original naming of these as mucopolysaccharides. As essential components of cell surfaces, GAGsplay an important role in mediating cell-cell signaling & adhesion. There are 6 major classes of GAGs, includingchondroitin 4- & 6-sulfates, keratan sulfate, dermatan sulfate, heparin, heparan sulfate & hyaluronic acid. All of theGAGs, except hyaluronic acid, are found covalently attached to protein, forming proteoglycan monomers, whichconsist of a core protein to which the linear GAG chains are covalently attached. The proteoglycan monomersassociate with a molecule of hyaluronic acid to form proteoglycan aggregates. GAGs are synthesized in the ER &Golgi. The polysacch chains are elongated by sequential addition of alternating acidic & amino sugars, donated bytheir UDP- derivatives. The last step in synthesis is the sulfation of some of the amino sugars. The source of thesulfate is 3’- phosphoadenosyl-5`-phosphosulfate. • GAGs are degraded by lysosomal hydrolases. They are 1st brokendown to oligosacch’s, which are degraded sequentially from the non-reducing end of each chain. A deficiency of oneof the hydrolases results in a mucopolysaccharidoses. These are hereditary disorders in which GAGs accumulate intissues, causing symptoms such as skeletal & extracellular matrix deformities, & mental retardation. Examples ofthese genetic diseases include Hunter & Hurler syndromes. Glycoproteins are proteins to which oligosacch’s arecovalently attached. They differ from proteoglycans in that length of glycoprotein’s CHO chain is relatively shortusually 2-10 sugar residues long. The CHO’s of glycoproteins do not have serial repeats as do GAGs. Memb-boundglycoproteins participate in a broad range of cellular phenomena, including cell surface recognition (by other cells,hormones, viruses), cell surface antigenicity (e.g., blood group antigens), & components of the extracellular matrix &of the mucins of the GI & urogenital tracts, where they act as protective biologic lubricants.

Keywords: GAGs, Glycoproteins, cell surface recognition.

Overview

They are most abundant organic molecules innature which provide: (i)important part of energyin diet. (ii)Act as the storage form of energy in thebody. (iii)structural component of cell membrane

The empiric formula is (CH2O)n – “hydrates ofcarbon”

Polysaccharides: more than 10 sugar units Theyare Homopolysaccharides andheteropolysaccharides

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Oligosaccharides : 3-10 monosaccharide units Disaccharides : 2 monosaccharide units Monosaccharides : Simple sugar

CLASSIFICATION

Monosaccharides Further classification is basedon: 1. No. of carbon atoms 2. Functional group:Aldehyde group – aldoses Keto group – ketoses

Joining of 2 monosaccharides by O-glycosidicbond: Maltose (α-1, 4) = glucose + glucoseSucrose (α-1, 2) = glucose + fructose Lactose (β-1, 4) = galactose + glucoseDisaccharides

Carbohydrates attached to non-carbohydratestructures by glycosidic bonds (O- or N-type) e.g.1. Purine and pyrimidine bases in nucleic acids 2.

Aromatic rings in steroids 3. Proteins inglycoproteins and glycosaminoglycans 4. Lipidsfound in glycolipids 5. Bilirubin

Complex CarbohydratesO-GlycosidicN-GlycosidicGlycosidic Bonds

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D-Mannitol 2. Sugar Acids for example (i)Gluconic acid derive from oxidation of C1ofglucose. (ii) D-Glucoronic acid derived fromoxidation of C6 of Glucose. (iii) L-Iduronic acidderived from oxidation of C6 of Glucose. D-Glucoronic acid and L-Iduronic acid are opticalisomers. D-Dulcitol (iii) D-Mannose D-Sorbitol (ii) D-Galactose Important Derivativeof Monosachhrides 1. Sugar Alcohols for example(i) D-Glucose

4. Amino Sugars (i) D-Glucosamine (ii) D-Galactosamine (iii) N-Acetylneuraminic acid(NANA) is an important component ofGlycolipids and Glycoproteins.

Heteropolysaccharides: e.g. glycosaminoglycans(GAGs) Homopolysaccharides: Branched : glycogen andstarch (α-glycosidic polymer) Unbranched :cellulose (β-glycosidic polymer)Polysaccharides

HOMOPOLYSACCHARIDES:

Glycogen • also known as animal starch • storedin muscle and liver (mostly) • present in cells asgranules (high MW) • contains both a(1,4) linksand a(1,6) branches at every 8 to 12 glucose unit(more frequent than in starch) • completehydrolysis yields glucose • glycogen and iodinegives a red-violet color • hydrolyzed by both aand b-amylases and by glycogen phosphorylase.

HOMOPOLYSACCHARIDES:

Inulin • b-(1,2) linked fructofuranoses • linearonly; no branching • lower molecular weight thanstarch • colors yellow with iodine • hydrolysisyields fructose • sources include onions, garlic,

dandelions and jerusalem artichokes • used asdiagnostic agent for the evaluation of GFR (Renalfunction test).

Jerusalem artichokes

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HOMOPOLYSACCHARIDES:

Chitin • chitin is the second most abundantcarbohydrate polymer • Like cellulose, chitin is astructural polymer • present in the cell wall of

fungi and in the exoskeletons of crustaceans,insects and spiders • chitin is used commerciallyin coatings (extends the shelf life of fruits andmeats) • A chitin derivative binds to iron atoms inmeat and slows the rancidity process

Sulfate groups GAGs are strongly negatively-charged: carboxyl groups of acidic sugars Theacidic sugar is either D-glucuronic acid or L-iduronic acid The amino sugar (usually sulfated)

is either D-glucosamine or D-galactosamine GAGs are linear polymers of repeatingdisaccharide units [acidic sugar-amino sugar]nGlycosaminoglycans (GAGs)

Resilience of GAGs • Being negatively chargedGAG chains are extended in solution and repeleach other and when brought together, they "slip"past each other • This produces the "slippery"consistency of mucous secretions and synovialfluid • When a solution of GAGs is compressed,the water is "squeezed out" and the GAGs are

forced to occupy a smaller volume. When thecompression is released, the GAGs spring back totheir original, hydrated volume because of therepulsion of their negative charges This propertycontributes to the resilience of synovial fluid andthe vitreous humor of the eye

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Members of GAGs Examples of GAGs are: 1.Chondroitin sulfates 2. Keratan sulfates 3.Hyaluronic acid 4. Heparin 5. Heparan Sulphate6. Dermatan Sulfate

CHONDROITIN SULFATES

1. They are Disaccharide units of Sulfated N-acetylgalactosamine + Glucuronic acid 2. It

contains N-AC Galactosamine in stead of N-acetyl glucosamine. 3. Most abundant GAG in thebody. 4. Form proteoglycan aggregates. 5. It ispresent in the ground substance of connectivetissues distributed in cartilage, bone, tendons,cornea and skin. 6. In cartilage, they bind collagenand hold fibers in a tight, strong network.

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KERATAN SULFATES 1. It is the only GAGwhich does not contain any uronic acid. 2.Sulfatecontent is variable and may be present on C-6 ofeither sugar

KERATAN SULPHATE 3.Most heterogeneousGAGs. 4.Only N-Acetylglucosamine & Galactoseform repeating units of disaccharide. 5. It is foundin cornea, Loose CT and tendons.

HYALURONIC ACID

• Disaccharide unit: N-acetylglucosamineGlucuronic acid • Different from other GAGs:they are unsulfated. Not covalently attached to

protein The only GAG found in bacteria. • Servesas a lubricant and shock absorber. • Found insynovial fluid of joints, vitreous humor of the eye,the umbilical cord, and cartilage.

HYALURONIC ACID

1. Present in connective tissue, tendons, synovialfluid, vitreous humor, umbilical cord & cartilage.2. Forms ground substance in combination withproteins. 3. Hyaluronidase is an enzyme present intesticular tissue and spleen. It catalyzesdepolymerization of hyaluronic acid and reducesits viscosity which defuses into the tissue spaces.So it is known as spreading factors. 4. It dissolvethe viscid substance around the ova to permit

penetration of spermatozoa. 5. Clinically it is usedto increase the efficiency of absorption ofsolutions administered by clysis.

HEPARIN 1. Disaccharide unit of Glucosamineand Glucuronic or iduronic acids 2. Sulfate isfound on glucosamine and uronic acid. 3. Unlikeother GAGs that are extracellular, heparin is anintracellular component of mast cells that linearteries, especially liver, lungs and skin.

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HEPARIN 4. It activates antithrombin III, whichin turn inactivates thrombin, factor X and factorIX. 5. It is an anticoagulant used for taking bloodfor biochemistry studies. 6. Used in suspectedthrombo embolic conditions to preventintravascular coagulation.

HEPARAN SULPHATE 1. They arecomponents of plasma membrane of the cells theyact as receptors and can help in the cell- cellinteractions. 2. Chondroitin Sulphate andHyaluronic acid are present in high concentration

in cartilages and help as cushion in the weightbearing joints.

DERMATAN SULFATE

1, 3 linkages. 2. Present in skin, blood vessels andheart valves. 3. This is present in the Sclera of theeye helps in maintaining the shape of theeye.DERMATAN SULPHATE 1. It contains L-iduronic acid and N-acetyl galactosamine in

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Proteoglycans and glycoproteins are wo kinds ofglycoconjugates that contain protein. 7P2-37Glycoconjugates are compounds that covalentlylink carbohydrates to proteins and lipids.Glycoconjugates

Proteoglycans • Proteoglycans have a very highcarbohydrate to protein ratio, often 95:5, and arefound in the extracellular matrix. • GAG chainsare linked to core proteins by N- and O-glycosidic links. 7P2-38

Proteoglycans

Glycoproteins • These contain carbohydrateresidues on protein chains. • Very importantexamples of these materials are antibodies-chemicals which bind to antigens and immobilizethem. • The carbohydrate part of the glycoproteinplays a role in determining the part of the antigenmolecule to which the antibody binds. 7P2-40

Glycoproteins: The human blood groups A, B,AB, and O depend on the oligosaccharide which

form part of the glycoprotein on the surface oferythrocyte cells. The terminal monosaccharide ofthe glycoprotein at the nonreducing enddetermines blood group. 7P2-41

Glycoproteins: 3 Type Terminal sugar A N -acetylgalactosamine B a - D-galactose AB boththe above O neither of the above O is the“universal donor” AB is the “universal acceptor”

Glycoproteins:

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Premature death in severe forms. variabledegrees of progressive mental and physicaldeterioration. excessive urinary excretion ofGAGs, intralysosomal accumulation of GAGs,Mucopolysaccharidosis 1. MPS are inheritablestorage diseases caused by a efficiency oflysosomal enzymes that degradeglycosaminoglycans (GAGs, previously calledmucopolysaccharides). 2. There is excessiveaccumulation of Grannules of MPS in bodytissue. 3. The MPSs are a heterogeneous groupcharacterized by the:-

MucopolysaccharidosIs 1.Pathogenesis/Pathology comprises:- • a specificlysosomal enzyme deficiency. • and many havevariable phenotypic expression. 2. characteristicdegrees of organ involvement and rates ofdeterioration.

MucopolysaccharidosIs . 3. Depending on theenzyme deficiency, the metabolism of:- •dermatan sulfate, heparan sulfate, keratan sulfatemay be blocked. • Lysosomal accumulation of theGAGs eventually results in cell, vascular, tissue,and organ dysfunction

MucopolysaccharidosIs • Main disturbances are:-• Inability to breakdown mucopolysaccharides •Lack or deficiency of enzyme • Inherited • Lackof gene

Mucopolysaccharidosis Results: • many seriousphysical disorders • Various genetic deformitiessuch as: • skeletal deformities (especially of the

face) • mental retardation • decreased lifeexpectancy

Mucopolysaccharidoses Hurler syndrome Huntersyndrome Sanfilippo syndrome Morquio diseaseMaroteaux-Lamy syndrome SLY Syndrome VIIScheie syndrome

Mucopolysaccharidosis • Mucopolysaccharidosesare hereditary disorders that are clinicallyprogressive. They are characterized byaccumulation of GAGs in various tissues, causingvaried symptoms, such as skeletal & extracellularmatrix deformities, & mental retardation. •Mucopolysaccharidoses are caused by adeficiency of one of the lysosomal hydrolasesnormally involved in degradation of heparansulfate and/or dermatan sulfate.

• This results in the presence of oligosacch’s inurine, because of incomplete lysosomaldegradation of GAGs. These fragments can beused to diagnose the specificmucopolysaccharidosis, namely by identifying thestructure present on the non- reducing end ofoligosacch. That residue would have been thesubstrate for the missing enz. • Diagnosis can beconfirmed by:- 1. measuring the patients cellularlevel of lysosomal hydrolases. 2. Children whoare homozygous for one of these diseases areapparently normal at birth, then graduallydeteriorate. 3. In severe cases, death occurs inchildhood. • All of the deficiencies are autosomal& recessively inherited except Hunter syndrome,which is X-linked.

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Bone marrow transplants are currently being usedsuccessively to treat Hunter syndrome; thetransplanted macrophages produce the sulfataseneeded to degrade GAGs in the extracellularspace. Note: some of lysosomal enzymes requiredfor degradation of GAGs also participate indegradation of glycolipids & glycoproteins.Therefore, an individual suffering from a specificmucopolysaccharidosis may also have a lipidosisor glycoprotein-oligosaccharidosis

Hurler syndrome(gargoylism) type I (Alpha-L-iduronate deficiency )

- L-Iduronidase accompanied by degradation ofdermatan sulphate and heparan sulphate. • Due todeposition of the faulted degradation products incoronary artery leads to ischemia and early death.• Severty varies from mild to most severe. •

Storage of abnormal quantities of this material(mucopolysaccharide) in different body tissues isresponsible for the symptoms and appearance ofthe disease.Hurler syndrome (gargoylism) type IDefinition • It is an inherited disorder withdeficiency of

Hurler syndrome(gargoylism) type I CONT. •Manifestation of the disease is by: 1. Cornealclouding 6. Heart disease 2. Mental retardation 7.Dysostosis multiplex 3. Dwarfing 8.Hepatosplenomegaly 4. Coarse facial features 9.Cardiac disease 5. Upper airway obstruction 10.Death before 10 yr of age 1. It can be treated bybone marrow or cord blood transplantation. 2.Enzyme replacement therapy is also available. 3.The child must be treated before 18 months ofage.

Hurler syndrome (type I)

Mucopolysaccharidosis I (MPS I) Disease (Hurler, Hurler-Scheie, Scheie Syndromes)

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SYMPTOMES IMAGES OF KEYFEATURES Hernia corneal clouding Coarsefacial Claw hand

Causes of the Hurler syndrome • Inherited as anautosomal recessive trait. • Metabolic defect:inability of the body to make an enzyme. • Thebody's to make an enzyme: lysosomal alpha-L-iduronase

Incidence & and risk factors Cont. •Approximately 1 in 150,000 infants are affected. •Newborn infants with this defect appear normal atbirth. • By the end of the first year, signs ofimpending problems begin to develop.

MPS (Type I) . • The children slowly develop. •Coarse, thick, facial features • Prominent darkeyebrows • Progressive stiffness • Mentalretardation

Prevention • Genetic counseling: important forparents with a family history of Hurler syndrome• Prenatal diagnosis. • An amniocentesis in theamniotic fluid are then cultured and the a-L-iduronidase activity in the cells is determined.

Symptoms . • Short stature • Severe mentalretardation • Thick, coarse facial features withlow nasal bridge • Full lips with a thick, largetongue • Increased body hair (hirsutism)

Symptoms . • Umbilical hernia • Deafness •Stiffness (in joints) • Shortness of breath •Abnormal bones of spine and claw hand

MPS: Signs . • Hepatomegaly • Splenomegaly •Enlarged tongue • Retinal pigmentation • Hipdislocation • Kyphosis • Heart murmurs • Heartvalve damage from thickening

Tests that may indicate the syndrome Cont. •Increased excretion of dermatan sulfate andheparan sulfate in the urine. • Absence oflysosomal alpha-L-iduronidase (in culturedfibroblasts). • Culture of cells from amniotic fluidobtained by amniocentesis for enzyme testing(prenatal testing).

Tests that may indicate the syndrome Cont. •Abnormal histological staining of white bloodcells called metachromasia • X-ray of the skeleton• X-ray of the spine • X-ray of the chest • ECG

RADIOLOGY depicts:- • Large skull, withthickened calvarium, • shallow orbits, • Abnormalspacing of teeth with dentigerous cysts. •kyphosis. • The pelvis is usually poorly formed •The clavicles are short, thickened, and irregular. •The ribs have been described as oar-shaped. • Thephalanges are short and trapezoid with wideningof the diaphyses.

GENETICS • on chromosome 4p16.3 and spans19?kb and includes 14 exons. • Mutation analysishas revealed two major alleles, W402X andQ70X, and a minor allele, P533R, • homozygosityor compound heterozygosity give rise to Hurlersyndrome

Hunter syndrome type II (Sulpho-idoronidesulphatase deficiency )Hunter syndrome type IIHunter syndrome type II (Sulpho-idoronidesulphatase deficiency ) This is an X-linkedcongenital disorder characterised by deficiency ofIduronic Sulphatase comprises by:- • wide rangeof severity • No corneal clouding • Physicaldeformity is mild to severe • Facial features. •Progressive stiffness • Decreased mentaldevelopment

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• Hepatomegaly (liver enlargement) •Splenomegaly (spleen enlargement) • Abnormalbone x-rays Clinical features are:- • Coarse facialfeatures, • short stature, • skeletal deformities, •joint stiffness, • and mental retardation • withonset of disease usually between 2–4 yr of age •chronic diarrhea. • Communicating hydrocephalus

• Extensive, slowly progressive neurologicinvolvement usually precedes death • Deathusually occurs between 10–15 yr of age.GENETIC:- • The gene encoding IDS containsnine exons that span 24?kb and is mapped toXq28. • About 20% of patients with the severeform of MPS II have major deletions orrearrangements of the IDS gene.

Type D : N-Acetylglucosamine-6-sulfatasedeficiency. • These enzymes help the body get ridof a substance normally found outside of our cellscalled a mucopolysaccharide • Level of heparansulfate increased, and in Sanfilippo syndrome,large amounts of it are excreted in the urine TypeC : Glucosamine-N-acteyltransferase deficiency. Type B : N-Acetylglucosamindase deficiency. Type A : Heparan Sulfaminidase deficiencySanfilippo syndrome type III Definition •Sanfilippo syndrome is one of the hereditarymucopolysaccharide storage diseases • It ischaracterized by the absence of one of severalenzymes. Due to multiple enzyme deficiency thisdisease is of four types:

This is an autosomal recessive trait • It is possiblythe most common of the mucopolysaccharidestorage diseases • It has a relatively late onsetrather than during the first year of life Sanfilipposyndrome Causes

CLINICAL MANIFESTATION

. • Coarse, thick, facial features • Prominent darkeyebrows • Progressive stiffness • Gaitdisturbances • Speech disturbances • Decreasedmental development that progresses to severemental retardationAn amniocentesis in the amniotic fluid are thencultured and the enzyme activity in the cells isdetermined. Genetic counseling is advised to theprospective parents with a family history of

Sanfilippo syndrome Prenatal diagnosis:Prevention Cont.

Symptoms . • Family history of Sanfilipposyndrome. • May have normal growth during firstfew years, but final height is below average. •Delayed development followed by deterioratingmental status. • Deterioration of gait.

Symptoms . • Coarse facial features • Full lips •Heavy eyebrows that meet in the middle of theface above the nose. • Diarrhea • Stiff joints thatmay not extend fully.

Abnormal bone x-rays such as thickened skull andoval vertebrae Echocardiogram may showthickened heart Corneas clear Splenomegaly(spleen enlargement) Hepatomegaly (liverenlargement) Sanfilippo syndrome Signs andtests

Sanfilippo syndrome Signs and tests • Seizures •mental retardation • Activities of one of theenzymes may be low in fibroblast skin cells •Urine may have increased heparan sulfate •Abnormal pathological staining character of whiteblood cells called metachromasia

Features and Characteristics children withSanfilippo syndrome • Occasional enlarged head •Coarse facial features • Coarse hair • Excessivehair growth • Joint stiffness

Sanfilippo syndrome • Severe diarrhea orconstipation • Severe hearing loss • Hyperactivity• Aggressive and destructive behavior • Poorattention • Physical aggression • Speech andlanguage delay • Sleep disturbance

Vision impairment Mild growth retardation. Severe intellectual impairment most often before6 years of age. Sanfilippo syndrome (Cont.)

Morquio syndrome Type IV • Deficiency ofGalactose-6-sulfatase • Accumulation of Keratansulfate • Autosomal Recessive. CLINICALFEATURES • Both types of Morquio syndromeare characterized • by significant, short-trunkdwarfism, • fine corneal deposits,

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CLINICAL FEATURES

• a skeletal dysplasia that is distinct from othermucopolysaccharidoses, • and preservation ofintelligence. • The appearance of genu valgus, •kyphosis, • growth retardation with short trunkand neck, • and waddling gait with a tendency tofall are early symptoms of MPS IV. • Gene is onchromosome 16q24.3.

• Extraskeletal manifestations may include:- 1.mild corneal clouding, 2. hepatomegaly, 3.cardiac valvular lesions, 4. and small teeth withabnormally thin enamel and frequent cariesformation. DIAGNOSIS • Analysis of urinaryGAGs is an initial diagnostic test(semiquantitativespot test to more precise qualitative andquantitative) • definitive diagnosis established byenzyme assay ( Serum, leukocytes, or culturedfibroblasts) • Prenatal diagnosis is available for allMPSs and is carried out on cultured cells fromamniotic fluid or chorionic villus biopsy. •Molecular diagnosis is the preferred method ofcarrier testing provided that the mutation in thefamily under consideration is known.

TREATMENT

• Unrewarding • Symptomatic • Orthopedicprocedures, including femoral osteotomies,acetabular reconstruction, and posterior spinalfusion, are necessary • Bone marrow transplant. •Bone marrow transplantation has resulted insignificant clinical improvement of somaticdisease in MPS I and increased long- termsurvival. Resolution or improvements have beennoted in hepatosplenomegaly, joint stiffness,facial appearance, obstructive sleep apnea, heart

disease, communicating hydrocephalus, andhearing loss.

Morquio syndrome subtypes A & -Galactosidasedeficiency. • Autosomal recessive.B Type IV •Type IVA: Galactose-6- sulfatase deficiency. •Type IVB:

Features and Characteristics children withMorquio syndrome • Joint stiffness • Mild growthretardation • Without mental retardation ! •Abnormal x-rays of:- Bone Skeleton Spine Chest

PREVENTION

Genetic counseling: important for prospectiveparents with a family history of Morquiosyndrome Prenatal diagnosis: • An amniocentesisin the amniotic fluid are then cultured and theenzyme activity in the cells is determined.

Morquio's syndrome type IVMucopolysaccharidosis • presence of keratansulfate in the urine • short stature due to severedeformity of the spine and the thorax, long boneswith irregular epiphyses, enlarged joints, flaccidligaments, and a waddling gait • Type A:deficiency of the enzyme N- acetylgalactosamine-6-sulfate sulfatase. Type B: deficiency of theenzyme beta- galactosidase

Maroteaux-Lamy syndrome Type V (N-Acetyl-galactose-amin-4-sulfatase) (Arylsulfatase B)

synovial fluids mucous secretions body cells A gel-like substance found in: DefinitionCont.

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Coarse facial features (N-Acetyl-galactose-amin-4-sulfatase (Arylsulfatase B) These are leading

features.Features and Characteristics Maroteaux-Lamy syndrome

Treatment . • At the present time, there is no curefor MPS disorders. • Enzyme replacement therapyand gene therapy are coming up as twotreatments. • Research for suitable treatment isgoing on invarious countries.

mental deficiency Corneal clouding Shortstature Skeletal deformity Splenomegaly

Hepatomegaly - glucuronidase. • There isdefective degradation of Dermatan sulfate andheparan sulfate. • The disease is characterised by :SLY SYNDROME (MPS VII) • In this diseasethere is congenital deficiency of Mild mentalretardation

Skeleton general dysplasia of the coarseningof the facies progressive corneal clouding,Scheie's syndrome Type I SMucopolysaccharidosis • alpha-L iduronidase

defect • high levels of chondroitin sulfate B in theurine .Scheie’s Syndrome • Lysosomal alpha-L-

iduronidase • Mildest • Autosomal recessive

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How to cite this article: Anil Batta. (2016). Medical aspects of Carbohydrates: Heteropolysaccharides.Int. J. Curr. Res. Med. Sci. 2(1): 65-78.

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