Biochemistry LMR - Dr.
Mohammed Azam
Pathway
Glycolysis/Embden Mayerhoff
Pathway (EMP)
Krebs Cycle /TCA cycle /Citric Acid
Cycle
Gluconeogenesis
Glycogenesis
Glycogenolysis
HMP Shunt/ Pentose Phosphate
pathway
Urea cycle/Ornithine Cycle
Beta oxidation of fatty acid
Ketogenesis
Fatty acid Synthesis
Cholesterol Synthesis
Purine Synthesis
CONSISTENCY is mantra of SUCCESS - Dr Mohammed Azam
Disease
Type I/ Von Gierke's Disease
Type II/Pompe's Disease
Type III/Cori's Disease/Limit
Dextrinosis
Type IV/ Anderson's Disease
Type V/ Mc Arlde's Disease
Type VI/ Her's Disease
Essential Fructosuria
Fructose Intolerance
Site Rate limiting enzyme
CARBOHYDRATE METABOLISM
Cytosol Phosphofructokinase-I (PFK-I)
Mitochondrial Matrix Isocitrate Dehydrogenase
Liver & Kidney ( Cytosol & Fructose 1,6-bisphosphatase
Mitochondria)
Liver & Skeletal muscle (Cytosol) Glycogen Synthase
Liver & Skeletal muscle (Cytosol) Glycogen Phosphorylase
Cytosol Glucose-6-Phosphate dehydrogenase
(G6PD)
PROTEIN METABOLISM
Liver (Cytosol & Mitochondria) Carbamoyl Phosphate Synthase-I
(CPS-I)
LIPID METABOLISM
Mitochondria Carnitine Acyl Transferase-I (CAT-I)
Mitochondria HMG CoA Synthase
Cytosol Acetyl CoA Carboxylase
Cytosol HMG CoA Reductase
NUCLEOTIDE METABOLISM
PRPP glutamyl Amidotransferase
Enzyme Deficient
Carbohydrates
Glucose-6-phosphatase SEVERE Hypoglycemia, Hepatomegaly &
Renomegaly, Lactic acidosis,
Hyperlipidemia, Ketosis and Hyperuricemia
(Gout).
Acid Maltase/ Lysosomal glucosidase Cardiomyopathy, hypotonia & systemic
findings lead to early death by 2 year.
Debranching Enzyme Similar to TYPE-I but milder symptoms &
normal blood lactate levels.
Branching Enzyme Hepatosplenomegaly, failure to thrive in
early infancy & progressive cirrhosis (death
usually before 5th year).
Muscle Glycogen Phosphorylase Painful muscle cramps with strenuous
exercise & muscle glycogen abnormally
high.
Hepatic Glycogen Phosphorylase Hepatomegaly.
Fructokinase Fructose appears in blood & urine.
Aldolase B Jaundice, vomiting, seizures, lethargy.
Hepatomegaly.
Galactossemia Galactose-1-phosphatase uridyl Jaundice, vomiting, seizures, lethargy.
transferase (GALPUT) Hepatomegaly & Oil drop cataract.
Mental retardation.
Protein's

Phenylketonuria (Mousy/Musty Phenylalanine Hydroxylase Hypopigmented skin, Blonde hairs,

odour) Intellectual disability, microcephaly, seizures
& mousey or musty odour.
Alkaptonuria /Black Urine Disease Homogentisate Oxidase Urine turns black on prolonged exposure to
air; Ochronosis (bluish-black connective
tissue, ear cartilage & sclerae.
Maple Syrup Urine Disease (Burnt Branched Chain Keta Acid Vomiting, poor feeding, urine smells like
Sugar Odour) Dehydrogenase/Decarboxylase maple syrup/burnt sugar.
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 Hyperuricemia & Gout, Intellectual
disability, Self mutilation and red/orange
crystals in urine.
Gout Hyperuricemia Needle shaped MonoSodium Urate (MSU)
Crystals and Gouty arthritis.
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), Creatine
Glycine Heme, Purine ring, Glutathione, Creatine

Inhibitor
Flouride (NaF) Enolase ( Glycolysis)
Barbiturates(PhenobarbitONE) and RotenONE Complex-I (ETC)
Malonate Complex-II (ETC)
Antimycin, British Anti Lewisite (BAL) Complex-III (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

Fat Soluble Vitamins

Vitamin Chemistry Function Deficiency
Vit-A Retinol Vision ( Rhodopsin : Opsin Nyctalopia
Retinal + 11Cis Retinal) Xerophthalmia
Retinoic Acid Normal Reproduction Keratomalacia
Normal differentiation of Dry & scaly skin
Epithelium Bitot spots (Keratin debris;
Antioxidant foamy appearance on
conjunctiva)
 Vit- D
Vit-E
Vit-K
Vitamin
Vit-B1 ( Thiamine )
Vit-B2 (Riboflavin)
Vit-B3 ( Niacin/Nicotinic
acid)
Vit-B5 ( Pantothenic Acid )
Vit-B6 ( Pyridoxine )
Vit- B7 ( Biotin/Vit-H)
Vit-D1(Calciferol) Intestinal absorption of
Vit-D2 (Ergocalciferol) calcium and phosphate.
Vit-D3 (Cholecalciferol) Bone mineralization.
Reabsorption of calcium&
phosphate from kidney.
Tocopherol Antioxidant
Vit-K1 (Phylloquinone) Activated form acts as
Vit-K2 (Menaquinone) cofactor for the
Vit-K3 (Menadione) γ-carboxylation of
Glutamate for clotting
factors II,VII,IX, X and
Protein C&S.
Water Soluble Vitamins
Cofactor Enzyme
Thiamine Pyrophosphate Pyruvate
(TPP) Dehydrogenase(PDH)
α-Ketoglutarate
dehydrogenase
Branched chain ketoacid
dehydrogenase (BCKD)
Transketolase
FAD & FMN Dehydrogenases
NAD & NADP Dehydrogenases
CoA ( Coenzyme A ) - Burning feet Syndrome,
Pyridoxal Phosphate (PLP) Transamination (ALT&AST)
Heme synthesis ( ALA
Synthase)
Decarboxylation reactions.
Glycogen Phosphorylase.
Biotin Pyruvate carboxylase
Propionyl CoA carboxylase
Acetyl Carboxylase
Rickets in children
Osteomalacia in adults
Neurological disorders
Hemolytic anemia
Neonatal hemorrhage with
increased PT & aPTT but
normal bleeding time.
Deficiency
Beri-Beri
Wernicke-Korsakoff
Syndrome
Chielosis (inflammation of
lips, scaling and fissures at
the corners of the mouth)
Magenta tongue
Seborrheic dermatitis
Pellagra
- Dementia
- Diarrhea
- Dermatitis (Casal’s
Necklace)
dermatitis, alopecia
Sideroblastic Anemia
Conculsions
Peripheral Neuropathy
Alopecia
Caused by long term
antibiotic use or excessive

Vit- B12 ( Cobalamin)
Vit – B9 ( Folic Acid )
Vit – C (Ascorbic Acid)
ingestion of raw egg
whites(AVIDIN in egg
whites avidly binds biotin)
Vit-B12 Homocysteine methyl Megaloblastic Anemia
transferase (HMT) Peripheral Neuropathy
Methyl malonyl CoA Inc. serum Homocysteine
Mutase (MMC Mutase) and Methylmalonic acid.
Converted to tetrahydrofolate (THF), which is important for Megaloblastic Anemia
synthesis of nitrogenous bases in DNA & RNA Neural Tube Defects
Inc. serum Homocysteine.
Antioxidant SCURVY (swollen gums,
Iron absorption poor wound healing, easy
Post translational modification of collagen bruising, petechiae,
(Hydroxylation of Proline and Lysine ) hemarthrosis, corkscrew
hair)

-Nucleosome is formed by DNA looped around histone

octamer(H2A, H2B, H3 & H4).
- H1 binds to linker DNA.
-DNA has negative charge from phosphate group.
-Histones are positive charge from lysine and arginine.

Nucleosomiz

ET

Pathological 2,4DNP Aspirin

TUncouplett
Physiological Thermogybownfa
Thyroxine
FreeFattyAcid
Bilirubin
 EnzymeInhibition

Boat BATE

Isoenzymes

tRNAs

Click the links below to follow us on:

YouTube

Telegram

Instagram

Facebook