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Biochemistry LMR - Dr.

Mohammed Azam

Pathway Site Rate limiting enzyme


CARBOHYDRATE METABOLISM
Glycolysis/Embden Mayerhoff Cytosol Phosphofructokinase-I (PFK-I)
Pathway (EMP)

Krebs Cycle /TCA cycle /Citric Mitochondrial Matrix Isocitrate Dehydrogenase


Acid Cycle

Gluconeogenesis Liver & Kidney ( Cytosol & Fructose 1,6-bisphosphatase


Mitochondria)
Glycogenesis Liver & Skeletal muscle (Cytosol) Glycogen Synthase
Glycogenolysis Liver & Skeletal muscle (Cytosol) Glycogen Phosphorylase
HMP Shunt/ Pentose Phosphate Cytosol Glucose-6-Phosphate
pathway dehydrogenase (G6PD)
PROTEIN METABOLISM
Urea cycle/Ornithine Cycle Liver (Cytosol & Mitochondria) Carbamoyl Phosphate Synthase-I
(CPS-I)
LIPID METABOLISM
Beta oxidation of fatty acid Mitochondria Carnitine Acyl Transferase-I (CAT-
I)
Ketogenesis Mitochondria HMG CoA Synthase
Fatty acid Synthesis Cytosol Acetyl CoA Carboxylase
Cholesterol Synthesis Cytosol HMG CoA Reductase
NUCLEOTIDE METABOLISM
Purine Synthesis PRPP glutamyl Amidotransferase
CONSISTENCY is mantra of SUCCESS - Dr Mohammed Azam

Disease Enzyme Deficient


Carbohydrates
Type I/ Von Gierke's Disease Glucose-6-phosphatase
Type II/Pompe's Disease Acid Maltase
Type III/Cori's Disease Debranching Enzyme
Type IV/ Anderson's Disease Branching Enzyme
Type V/ Mc Arlde's Disease Muscle Glycogen Phosphorylase
Type VI/ Her's Disease Hepatic Glycogen Phosphorylase
Essential Fructosuria Fructokinase
Fructose Intolerance Aldolase B
Galactossemia Galactose-1-phosphatase uridyl transferase
Protein's
Phenylketonuria (Mousy/Musty odour) Phenylalanine Hydroxylase
Alkaptonuria /Black Urine Disease Homogentisate Oxidase
Maple Syrup Urine Disease (Burnt Sugar Odour) Branched Chain Keta Acid
Dehydrogenase/Decarboxylase
Lipid Storage Diseases
Niemann Pick Disease Sphingomyelinase
Gaucher's Disease Beta-Glucocerebrosidase
Krabbe's Disease Beta- Galactocerebrosidase
Fabry's Disease Alpha- Galactocerebrosidase
Tay Sach's Disease Hexosaminidase A
Sand Hoff's Disease Hexosaminidase A & B
Nucleotide's
Lesch Nyhan Syndrome HGPRT
Gout Hyperuricemia

CONSISTENCY is mantra of SUCCESS - Dr Mohammed Azam

Amino Acid Special Product


Tyrosine Melanin, T3, T4, Catecholamines
Tryptophan Melatonin, Serotonin, Vit B3
Histidine Histamine
Arginine Nitric Oxide (NO)

Inhibitor
Flouride Enolase ( Glycolysis)
Barbiturates(Phenobarbitone) and Rotenone Complex-I (ETC)
Malonate Complex-II (ETC)
CO, CN, H2S Complex-IV (ETC)
Oligomycin Complex-V (ETC) - ADP to ATP Conversion

Classification of Amino Acids


Aliphatic Amino Acid Glycine, Alanine, Valine, Leucine, Isoleucine
Hydroxyl group containing Amino Acid Serine, Threonine
Sulphur containing Amino Acid Cysteine, Methionine
Acidic Amino Acid Aspartic Acid, Glutamic Acid, Asparagine,
Glutamine
Basic Amino Acid Lysine, Arginine, Histidine
Aromatic Amino Acid Tyrosine, Tryptophan, Phenylalanine
Imino Acid Proline
Essential Amino Acid Tryptophan, Valine, Threonine, Isoleucine,Leucine,
Lysine, Phenylalanine, Methionine (TV TILL 8 PM)
Semi Essential Amino Acid Arginine, Histidine
Ketogenic Amino Acid Leucine, Lysine
Glucogenic and Ketogenic Amino Acid Tyrosine, Tryptophan, Phenylalanine, Isoleucine
Glucogenic Amino Acid Rest All
21st Amino Acid Selenocysteine (UGA)
22nd Amino Acid Pyrrolysine (UAG)
Fatty Acids
Essential Fatty Acids Linoleic Acid, Linolenic Acid, Arachidonic Acid

Miscellaneous
1. Uncouplers - 2,4-DNP, Aspirin.
(Physiological Uncouplers- Thermogenin, Thyroxine)
2. Lipoproteins with highest cholesterol - LDL.

3. Lipoprotein with highest triglyceride - Chylomicron.

4.Nature of Enzymes - Apoenzyme + Coenzyme = Holoenzyme

5. Competitive Inhibition- Vmax Same and Km increases.

6. Non Competitive Inhibition - Km Same and Vmax decreases.

7. Cofactors
Mineral Function
Cobalt Constituent Vitamin B12
Chromium Potentiate the action of insulin
Flouride Constituent of bone and teeth
Iodine Thyroid Hormone Synthesis
Molybdenum Cofactor for Xanthine Oxidase

Selenium Cofactor for Glutathione Peroxidase and Deiodinase

Zinc Cofactor for


Carbonic Anhydrase
Lactate dehydrogenase
Alcohol dehydrogenase
Alkaline Phosphatase

Manganese Cofactor for


Arginase
Carboxylase
Kinase
Enolase

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-:Compiled By :-
Dr. Mohammed Azam
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Fb group: ANATOMY-DR AZAM
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