carbohydrate chemistry 15

Carbohydrate Chemistrylecture 1

Prof. Aparna Misra

Biochemistry

Learning ObjectivesCarbohydrate Chemistry (Lecture 1)

By the end of the lecture, the student should know: Definition of carbohydrates.ClassificationMonosacharide,Properties & classificationThe functions and biological importance of monosaccharides

Definition

Poly hydroxy Aldehyde or poly hydroxy ketones & the substances that will yield such substances on hydrolysis.

Carbohydrates Contain the Elements:CarbonHydrogenOxygen They Are Split Into Three Groups Known As:Monosaccharides(Monomers) Disaccharides(Dimers)Polysaccharides(Polymers)

Many carbohydrates are soluble in water.

The usual chemical test for the simpler carbohydrates is heating with Benedicts solution.

The formula for a carbohydrate is (CH2O)n

The n represents the number of times the CH2O unite is repeated.

Biological Importance

Energy source.

Structural component.

Synthetic property.

Storage function.

. ROLE OF CARBOHYDRATES• As a major energy source for living organisms

(glucose is a principal energy source in animal and plants)

• As a means of transporting energy ( exp: sucrose in plant tissues)

• As a structural material ( cellulose in plants, chitin in insects, building blocks of nucleotides).

• As a precursor for other biomolecules (purine, pyrimide)

SIGNIFICANCE OF CARBOHYDRATES

• Carbohydrates are the most abundant bio-molecules in nature, having a direct link between solar energy and the chemical bond energy in living organisms.

• Source of rapid energy production• Structural building blocks of cells• Components of several metabolic pathways• Recognition of cellular phenomena, such as cell

recognition and binding (e.g., by other cells, hormones, and viruses)

Classification

• Monosaccharide.

• Disaccharide.

• Polysaccharides.

cellulose, chitin, cellulose, chitin, starch, glycogen, starch, glycogen, glucoaminoglycansglucoaminoglycans

disaccharidesdisaccharides GlycoproteinsGlycoproteins (bacterial cell (bacterial cell wallswalls

Carbohydrate

Mono Mono saccharidesaccharide

OligoOligosaccharidesaccharide

PolyPolysaccharidesaccharide GlyconoconjugatesGlyconoconjugates

Glucose, fructoseRibose (aldopentose)Deoxy ribose

glycoproteins and proteoglycans

Monosaccharide

Definition

• The simplest form carbohydrate that can not be hydrolyzed to simpler form.

• Example---Glucose.

COLORLESS, CRYSTALLINE SOLIDS

SOLUBLE IN WATER BUT INSOLUBLE IN NONPOLAR SOLVENTS

ONE OF THE CARBON ATOMS IS DOUBLE-BONDED TO AN OXYGEN ATOM TO FORM A CARBONYL GROUP; EACH OF THE OTHER CARBON ATOMS HAS A HYDROXYL GROUP.

CARBOHYDRATES WITH AN ALDEHYDE (-CHO) FUNCTIONAL GROUP ARE CALLED ALDOSES E.G. GLYCERALDEHYDE (CH2OH-CHOH-CHO)

THOSE WITH A KETO GROUP (-C=O) ARE KETOSES E.G.DIHYDROXYACETONE (CH2OH-C=O-CH2OH)

CLASSIFIED ACCORDING TO THE NUMBER OF CARBON ATOMS THEY CONTAIN

Monosacharides Properties& classification

Classification of monosaccharides

• According to number of carbon atoms– Trioses, Tetroses, Pentoses, hexoses, Heptoses & Octoses.

• According to functional group- Aldoses and ketoses.

SUMMARY OF SUGAR STRUCTURESISOMERS- compounds that have the same chemical formula e.g. fructose, glucose, mannose, and galactose are isomers of each other having formula C6H12O6.

EPIMERS- refer to sugars whose configuration differ around one specific carbon atom e.g. glucose and galactose are C-4 epimers and glucose and mannose are C-2 epimers.

ENANTIOMERS- a special type of isomerism found in pairs of structures that are mirror images of each other. The mirror images are termed as enantiomers and the two members are designated as D- and L- sugar. The vast majority of sugars in humans are D-sugars.

Stereoisomerism

• Carbon containing molecules with same chemical structure but different spatial arrangement.

• Asymmetric carbon atom--A carbon atom with 4 different groups.

• A molecule with one asymmetric carbon can have 2 stereoisomer.

D & L sugars

Found in a pair of sugars that are mirror images to each other.

They differ in orientation of H & OH groups in the penultimate carbon atom.

The reference molecules are D-glyceraldehydes.

Pyranoses & Furanoses.

• In aqueous solution common monosaccharides have cyclic structure.

• These cyclic structures will have an additional asymmetric carbon & can exist in two stereoisomeric forms.

• They are alpha & beta anomers.

Anomers• Isomers of monosaccharides that differs

only in their configuration around carbon number 1.

• Example-- alpha D & beta D Glucose.

Mutarotation

• Alpha &beta anomers of Glucose will inter convert in solution by a process called Mutarotation.

• A solution of alpha D & beta D glucose will form an equilibrium mixture of 1/3 alpha and 2/3 beta glucose.

Major Monosaccharides.

• Glucose.• Fructose.• Mannose.• Galactose.• Ribose.• Deoxyribose.• Erythrose.• Glyceraldehyde.• Dihydroxyacetone.

Monosaccharide derivatives.

• Sugar acids– Gluconic acid, Glucuronic acid, Gluccharic acid.

• Sugar alcohol.– Sorbitol, Mannitol, • Sugar phosphates.– Glucose 6 phosphate.• Deoxy sugar– Deoxyribose.• Amino sugar.– Hexosamines.• Sialic acid.– N acetyl neuraminic acid.

IMPORTANT REACTIONS INMONOSACCHARIDESIMPORTANT REACTIONS INMONOSACCHARIDES

Monosaccharides undergo the following Monosaccharides undergo the following reactions :reactions :

1.1.OxidationOxidation

2.2.ReductionReduction

3.3.IsomerizationIsomerization

4.4.EsterificationEsterification

5.5.Glycoside formationGlycoside formation

6.6.MutarotationMutarotation

IMPORTANCE OF MONOSACCHARIDES: GLUCOSE

THE STORAGE FORM OF GLUCOSE IN HUMANS IS GLYCOGEN

IN PLANTS IT IS STORED MAINLY IN THE FORM OF STARCH.

DIETARY SOURCES: FRUITS, VEGETABLES(IN THE FORM OF STARCH), HONEY

BIOLOGICAL SIGNIFICANCE

BRAIN CELLS, RBCS AND THE GROWING EMBRYO ONLY UTILIZE GLUCOSE AS A SOURCE OF ENERGY.

ENERGY SOURCE FOR CELLS IN THE BODY. BUILDING BLOCK OF DISACCHARIDES AND

POLYSACHHARIDES

IT IS THE SUGAR PRESENT IN BLOOD

IMPORTANT MONOSACCHARIDES• GLUCOSE • FRUCTOSE• GALACTOSE

D-Glucose:D-Glucose:D-glucose (dextrose) is the primary fuel in living cells especially in brain cells that have few or no mitochondria.

Cells such as eyeballs have limited oxygen supply and use large amount of glucose to generate energy

Dietary sources include plant starch, and the disaccharides lactose, maltose, and sucrose

DISORDERS ASSOCIATED WITH GLUCOSE

DIABETES MELLITUS

GLYCOSURIA

RENAL SUGAR THRESHOLD IT IS THE MAXIMUM CAPACITY OF KIDNEYS TO REABSORB GLUCOSE.

DIABETES (diabetes mellitus)1. Characterized by high blood glucose levels that splills

over into the urine2. These high glucose levels impairs the insulin-

stimulated glucose entry into cells and starve the cells of insulin.

3. This leads to ketosis or high levels of ketone bodies (acids) that hinders the buffering capacity of the blood in the kidney, which controls blood pH (by excreting excess H+ ions into the urine).

4. The H+ excretion is accompanied by the excretion ammonia, sodium,potassium, and phosphate ions causing severe dehydration

5. This leads to excessive thirst symptom of diabetes and life-threatening decrease in blood volume.

FRUCTOSE: IMPORTANCE AND BIOLOGICAL SIGNIFICANCE

DIETARY SOURCES: FRUIT JUICES, HONEY AND SUGAR CANE. SWEETEST SUGAR

SEMINAL FLUID IS RICH IN FRUCTOSE.

SPERM UTILIZES FRUCTOSE FOR ENERGY

IN THE SEMINIFEROUS TUBULAR EPITHELIAL CELLS, FRUCTOSE IS FORMED FROM GLUCOSE.

GALACTOSE: IMPORTANCE AND BIOLOGICAL SIGNIFICANCE

DIETARY SOURCE: DIARY PRODUCTS LESS SWEET THAN GLUCOSE USED IN THE SYNTHESIS OF MILK SUGAR IN

MAMMARY GLANDS IT IS A CONSTITUENT OF GLYCOLIPIDS AND

GLYCOPROTEINS IT IS REQUIRED FOR THE DEVELOPMENT OF

BRAIN AND NERVOUS TISSUE IN INFANTS.

MANNOSE

IT DOES NOT OCCUR FREE IN NATURE

IN THE HUMAN BODY, IT IS FOUND AS A CONSITUENT OF GLYCOPROTEINS

ITS REDUCTION PRODUCT THAT IS MANNITOL IS IMPORTANT CLINICALLY IN CEREBRAL EDEMA.

MONOSACCHARIDE DERVATIVES

• URONIC ACIDS – formed when terminal CH2OH group of a mono sugar is oxidised– Important acids in animals – D-glucuronic acid and

its epimer L-iduronic acid– In liver cells glucuronic acid combines with

steroids, certain drugs, and bilirubin to improve water solubility therby helping the removal of waste products from the body

– These acids are abundant in the connective tissue carbohydrate components.

Mono sugar derivatives

• AMINO SUGARS –– Sugars in which a hydroxyl group (common on

carbon 2) is replaced by an amino group e.g. D-glucosamine and D-galactosamine

– common constituents of complex carbohydrate molecule found attached to cellular proteins and lipids

– Amino acids are often acetylated e.g. N-acetyl-glucosamine.

Mono sugar derivatives

• DEOXYSUGARS – monosaccharides in which an - H has replaced an –

OH group– Important sugars: L-fucose (formed from D-

mannose by reduction reactions) and 2-deoxy-D-ribose

– L-fucose – found among carbohydrate components of glycoproteins, such as those of the ABO blood group determinates on the surface of red blood cells

– 2-deoxyribose is the pentose sugar component of DNA.

IMPORTANCE OF PENTOSES

RIBOSE: IT IS A CONSTITUENT OF NUCLEIC ACID THAT IS RNA

2-DEOXYRIBOSE: IT IS A CONSTITUENT OF DNA

GLYCOSIDES• Monosaccharides can be linked by glycosidic bonds (joining of

2 hydroxyl groups of sugars by splitting out water molecule) to create larger structures.

• Disaccharides contain 2 monosaccharides e.g. lactose (galactose+glucose); maltose (glucose+glucose); sucrose (glucose+fructose)

• Oligosaccharides – 3 to 12 monosaccharides units e.g. glycoproteins

• Polysaccharides – more than 12 monosaccharides units e.g. glycogen (homopolysaccharide) having hundreds of sugar units; glycosaminoglycans (heteropolysaccharides) containing a number of different monosaccharides species.

Disaccharides.Lactose – Galactose + Glucose.

• maltose– Glucose + Glucose.

• Sucrose – Glucose +Fructose.

DISACCHARIDES AND OLIGOSACCHARIDES

• Configurations: alfa or beta ( 1,4, glycosidic bonds or linkages; other linkages 1,1; 1,2; 1,3; 1,6)

• Digestion aided by enzymes. Defficiency of any one enzyme causes unpleasant symptoms (fermentation) in colon produces gas [bloating of cramps].

• Most common defficiency, an ancestoral disorder, lactose intolerance caused by reduced synthesis of lactase

Important sugars of Disaccharides • LACTOSE

(milk sugar) disaccharide found in milk; composed of one molecule of galactose and glucose linked through beta(1,4) glycosidic linkage; because of the hemiacetal group of the glucose component, lactose is a reducing sugar

LACTOSE

Also called milk sugar because it naturally occurs only in milk.

On hydrolysis it yields one molecule of glucose and one molecule of galactose which are linked together through 1-4 glycosidic linkage

Two Monomer Units of Lactose are:- Glucose. Galactose.

LACTOSE

Also called milk sugar because it naturally occurs only in milk.

On hydrolysis it yields one molecule of glucose and one molecule of galactose which are linked together through 1-4 glycosidic linkage

Two Monomer Units of Lactose are:- Glucose. Galactose.

Lactose intolerance• Lactose (milk sugar) in infants is hydrolyzed by

intestinal enzyme lactase to its component monosacch for absorption into the bloodstream (galactose epimerized to glucose).

• Most adult mammals have low levels of beta-galactosidase. Hence, much of the lactose they ingest moves to the colon, where bacterial fermentation generates large quantities of CO2, H2 and irritating organic acids.

• These products cause painful digestive upset known as lactose intolerance and is common in the African and Asian decent.

SUCROSE

It is common table sugar. Mainly found in Sugar Cane.

It has 1,2 glycosidic linkage. Two Monomer Units of Sucrose are:- Glucose. Fructose. CLINICAL SIGNIFICANCE: Small amounts of

oral sucrose placed in the infant's mouth reduces procedural pain.

MALTOSE

YIELDS UPON THE HYDROLYSIS OF STARCH(AMYLASE)

MADE UP OF TWO MOLECULES OF GLUCOSE GLYCOSIDIC LINKAGE(1,4) CLINICAL

SIGNIFICANCE: MALTASE IS DIGESTED BY THE ENZYME

MALTASE. BABY FOODS CONTAIN MALTOSE BECAUSE IT IS EASILY DIGESTED.

• CELLOBIOSE

degradation product of cellulose containing two molecules of glucose linked by a beta (1,4) glycosidic bond; it does not occur freely in nature

OLIGOSACCHARIDE SUGARS

• Oligosaccharides are small polymers often found attached to polypeptides in glycoproteins and some glycolipids.

• They are attached to membrane and secretory proteins found in endoplasmic reticulum and Golgi complex of various cells

• Two classes: N-linked and O-linked

Case study• A 20-year old man presents at medical OPD in

a hospital with a complaint of abdominal discomfort, diarrhoea and muscle cramps. From the case history, it appears that he had consumed a large quantity of milk and other diary product for three days on festive occasion. The house- surgeon who examined him , advised to avoid milk and milk products. The patient followed this advice and became normal in two days.

Glycosidic Bonds

• Hydroxyl group (OH) of anomeric carbon of a monosaccharide reacts with a hydroxyl group of another monosaccharide or a non-carbohydrate to form a glycosidic bond

• OH +H=H2O

PolysaccharidesComposed of large number of monosaccharide units connected by glycosidic linkage

Contain >10 monosaccharide units.Also called Glycans.

Polysaccharides are more hydrophobic if they have a greater number of internal hydrogen bonds, and as their hydrophobicity increases there is less direct interaction with water

Classification of Polysaccharides

Homopolysaccharides

• -made up of single variety of monosaccharide• - some acts as storage form of fuel• Found in abundance in nature• Important examples: starch, glycogen,

cellulose, and chitin

HOMOSACCHARIDES

• Starch, glycogen, and cellulose all yield D-glucose when they are hydrolyzed

• Cellulose - primary component of plant cells

• Chitin – principal structural component of exoskeletons of arthropods and cell walls of many fungi; yield glucose derivative N-acetyl glucosamine when it is hydrolyzed.

STARCH (Homosaccharide)• A naturally abundant nutrient carbohydrate, found chiefly in

the seeds, fruits, tubers, roots, and stem pith of plants, notably in corn, potatoes, wheat, and rice,

• Two polysaccharides occur together in starch: amylose and amylopectin

• Amylose – unbranched chains of D-glucose residues linked with alfa(1,4,)glycosidic bonds

• Amylopectin – a branched polymer containing both alfa(1,4,) and alfa(1,6) glcosidic linkages; the alfa(1,6) branch points may occur every 20-25 glucose residues to prevent helix formation

• Starch digestion begins in the mouth; alfa-amylase in the saliva initiates hydrolysis of the gycosidic linkages

Starch is digested by pancreatic amylasein the intestine

Amylase

•Starch → → Dextrins → Maltose and Isomaltose Maltase

→ → → Glucose → Absorbed through intestinalisomaltose

wall

Glycogen• Glycogen is the storage form of carbohydrate

in animal.• It is stored in muscle and in liver.• Composed of glucose units linked by α-(1-4)

and α-(1-6)glycosidic bonds• Similar to amylopectin except more branching

in glycogen• The interior core portion contains a primer

molecule known as glycogenin

CELLULOSE (Homosaccharide)• Cellulose is found in plants as microfibrils (2-20 nm

diameter and 100 - 40 000 nm long). These form the structurally strong framework in the cell walls.

• Cellulose is mostly prepared from wood pulp

• Cellulose is a linear polymer of β-(1 4)-D-glucopyranose units in 4C1 conformation. The fully equatorial conformation of β-linked glucopyranose residues stabilizes the chair structure, minimizing its flexibility

Cellulose has many uses as an anticake agent, emulsifier, stabilizer, dispersing agent, thickener, and gelling agent but these are generally subsidiary to its most important use of holding on to water.

Water cannot penetrate crystalline cellulose but dry amorphous cellulose absorbs water becoming soft and flexible.

Purified cellulose is used as the base material for a number of water-soluble derivatives e.g. Methyl cellulose, carbomethycellulose

CELLULOSE

ᵦ- amylase cellobiase

cellulose→ → →cellobiose → → →ᵦ-D-glucose

CHITIN (Homosaccharide)

• Chitin is a polymer that can be found in anything from the shells of beetlesto webs of spiders. It is present all around us, in plant and animal creatures.

• It is sometimes considered to be a spinoff of cellulose, because the two are very molecularly similar.

• Cellulose contains a hydroxy group, and chitin contains acetamide.

Chitin is unusual because it is a "natural polymer," or a combination of elements that exists naturally on earth.

Usually, polymers are man-made. Crabs, beetles, worms and mushrooms contain large amount of chitin.

Chitin is a very firm material, and it help protect an insect against harm and pressure

Structure of the chitin molecule, showing two of the N-acetylglucosamine units that repeat to form long chains in beta-

1,4 linkage.

CHITOSAN• This is a man-made molecule that is often used to dye

shirts and jeans in the clothing industry. • Chitosan can be used within the human body to

regulate diet programs, and researchers are looking into ways in which it can sure diseases.

• Chitin, the polysaccharide polymer from which chitosan is derived, is a cellulose-like polymer consisting mainly of unbranched chains of N-acetyl-D-glucosamine. Deacetylated chitin, or chitosan, is comprised of chains of D-glucosamine. When ingested, chitosan can be considered a dietary fiber.

CHEMICAL STRUCTURE OF CHITOSAN

HETEROPOLYSACCHARIDES

• Are high-molecular-weight carbohydrate polymers more than one kind of mono-saccharide

• Important examples include glycosaminoglycans (GAGs) – the principle components of proteoglycans and murein, a major component of bacterial cell walls.

GAGs - high-molecular-weight carbohydrate polymers

• Glycosaminoglycans forming the proteoglycans are the most abundant heteropolysaccharides in the body. They are long unbranched molecules containing a repeating disaccharide unit. Usually one sugar is an uronic acid (either D-glucuronic or L-iduronic) and the other is either GlcNAc or GalNAc. One or both sugars contain sulfate groups (the only exception is hyaluronic acid).

• GAGs are highly negatively charged what is essential for their function.

THE SPECIFIC GAGs OF PHYSIOLOGICAL SIGNIFICANCE

Hyaluronic acid Chondroitin sulphates Keratan sulphates Heparin and heparin sulphates Dermatan sulphate

Hyaluronic acid (lubricant)•

Occurence : synovial fluid, ECM of loose connective tissue

Hyaluronic acid is unique among the GAGs because it does not contain any sulfate and is not found covalently attached to proteins.

It forms non-covalently linked complexes with proteoglycans in the ECM.

Hyaluronic acid polymers are very large (100 - 10,000 kD) and can displace a large volume of water.

Hyaluronic Acid.

Alternating N Acetyl glucosamines & D Glucuronic acid in beta 1—3 linkage.

Present in synovial fluid of joints,

vitreous humor of eyes, cartilages & tendons.

Hyaluronidase –an enzyme present in some bacteria can hydrolyze H.A.

Chondroitin sulphate

• Glucuronic acid and N acetyl galactosamine in beta 1-3 linkage.

• Present in cartilages ,tendons, ligaments and walls of arteries.

Keratan sulphate 1&2

• Galactose & GalNac in beta 1-4 linkage.

• No uronic acid.

• Present in cornea, cartilage, bone, hair and nails.

Heparan sulphate

• Structurally similar to heparin but lower density of sulphate.

• Present in basement membrane of tissues and cell membrane.

Dermatan sulphate

• Iduronic acid and GalNac in beta 1—3 linkage.

• Present in skin ,blood vessels, cornea, sclera and heart valves.

• Responsible to maintain the shape of eyeball

Glycoconjugates

• Information- rich molecules• Play vital role in protein targeting(carrying newly synthesised proteins to their

destinations)

• Act as mediators of cell to cell interactions and communication between cells and extracellular matrix.

• Eg. Proteoglycans, glycoproteins and glycoconjugates

Proteoglycans.

• Gags joined covalently with proteins.

• Contains up to 95% of carbohydrate.

• Major component of connective tissue, for example cartilages.

Glycoprotein.

• Protein and oligosaccharide.

• Up to 70% carbohydrate.

• Immunoglobulins, Plasma protein, Hormones, Receptors , blood group substances are examples.

Glycolipids

Mostly occurs as gangliosides which occur in nervous tissues

Mucopolysaccharidoses.

• A group of hereditary diseases caused by disorders of proteoglycan metabolism.

• S\S---coarse facial features, thick skin, corneal opacity.

• Examples—Hurler’ syndrome, Hunter’ syndrome.

Genetic disorders

Deficiency of enzymes necessary to breakdown mucopolysaccharides (MPS)

Excessive accumulation of mucopolysaccharides in body tissues

MUCOPOLYSACCHARIDOSES

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Results:

many serious physical disorders Various genetic deformities such as:

skeletal deformities (especially of the face)mental retardation

decreased life expectancy

MUCOPOLYSACCHARIDOSES

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Hunter syndromeHurler syndromeScheie syndrome

Sanfilippo syndromeMorquio disease

Maroteaux-Lamy syndrome

EXAMPLES

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Definition

An inherited disease (AR)

Storage of abnormal quantities of this material

(mucopolysaccharide) in different body tissues is responsible for

the symptoms and appearance of the disease

HURLER SYNDROME TYPE I

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Hurler syndrome (type Hurler syndrome (type II((

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Hurler syndrome type IHurler syndrome type I

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Key Symptom Images

Hernia

Corneal clouding

Coarse facial features

Claw hand

Mucopolysaccharidosis I (MPS I( DiseaseMucopolysaccharidosis I (MPS I( Disease ((Hurler, Hurler-Scheie, Scheie SyndromesHurler, Hurler-Scheie, Scheie Syndromes((

Inherited as an autosomal recessive trait

Metabolic defect: inability The body's to make an enzyme:

lysosomal alpha-L-iduronase

CAUSES OF THE HURLER SYNDROME

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Approximately 1 in 150,000 infants are affectedNewborn infants with this defect appear normal at birth By the end of the first year, signs of impending problems begin to develop

INCIDENCE & AND RISK FACTORS

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The children slowly developCoarse, thick, facial features

Prominent dark eyebrowsProgressive stiffness Mental retardation

MPS (TYPE I)

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Genetic counseling: important for parents with a family history of Hurler syndrome

Prenatal diagnosis:An amniocentesis in the amniotic fluid are then cultured and the a-L-iduronidase activity in the cells is determined.

PREVENTION

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Short stature

Severe mental retardation

Thick, coarse facial features with low nasal bridge

Full lips with a thick, large tongue

Increased body hair (hirsutism)

SYMPTOMS

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Umbilical herniaDeafness

Stiffness (in joints) Shortness of breath

Abnormal bones of spine and claw hand

SYMPTOMS

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MPS: SIGNS

HepatomegalySplenomegaly

Enlarged tongue Retinal pigmentation

Hip dislocationKyphosis

Heart murmursHeart valve damage from thickening

Increased excretion of dermatan sulfate and heparan sulfate in the

urine

Absence of lysosomal alpha-L-iduronidase (in cultured fibroblasts)

Culture of cells from amniotic fluid

obtained by amniocentesis for enzyme testing (prenatal testing)

TESTS THAT MAY INDICATE THE SYNDROME

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Abnormal histological staining of white blood cells called

metachromasia X-ray of the skeleten

X-ray of the spineX-ray of the chest

ECG

TESTS THAT MAY INDICATE THE SYNDROME

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HUNTER SYNDROME TYPE II

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Hunter syndrome type IIHunter syndrome type II

X-linkedCoarse, thick, facial features

Progressive stiffness decreased mental development Hepatomegaly (liver enlargement) Splenomegaly (spleen enlargement)

Abnormal bone x-rays

HUNTER SYNDROME TYPE II (SULPHO-IDORONIDE SULPHATASE

DEFICIENCY )

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