Carbohydrate chemistry 15
-
Upload
draparna24 -
Category
Science
-
view
1.369 -
download
3
Transcript of 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
93
Results:
many serious physical disorders Various genetic deformities such as:
skeletal deformities (especially of the face)mental retardation
decreased life expectancy
MUCOPOLYSACCHARIDOSES
94
Hunter syndromeHurler syndromeScheie syndrome
Sanfilippo syndromeMorquio disease
Maroteaux-Lamy syndrome
EXAMPLES
95
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
96
97
Hurler syndrome (type Hurler syndrome (type II((
98
Hurler syndrome type IHurler syndrome type I
99
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
100
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
101
The children slowly developCoarse, thick, facial features
Prominent dark eyebrowsProgressive stiffness Mental retardation
MPS (TYPE I)
102
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
103
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
104
Umbilical herniaDeafness
Stiffness (in joints) Shortness of breath
Abnormal bones of spine and claw hand
SYMPTOMS
105
106
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
107
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
108
HUNTER SYNDROME TYPE II
109
110
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 )
111