glycine metabolism & its clinical significance

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Glycine metabolism Dr Rohini C Sane

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Page 1: Glycine metabolism & its clinical significance

Glycine metabolism

Dr Rohini C Sane

Page 2: Glycine metabolism & its clinical significance

Glycine metabolism

1) Non essential amino acids

2) Glucogenic amino acids

FUNCTIONS OF GLYCINE

AMMONIA GLUCOSE

BILE SALTS GLUTATHIONE

CREATINE HEME

DETOXIFICATION OXLATES

SERINE PROTEIN PURINE

GLYCINE

FUCTIONS OF Glycine : ABCDGGHOPPS

Page 3: Glycine metabolism & its clinical significance

Synthesis of Glycine From different biomolecules

Page 4: Glycine metabolism & its clinical significance

Glycine metabolism

1.Synthesis of Glycine from Serine

Page 5: Glycine metabolism & its clinical significance

Synthesis of Glycine from -SerineReversible reaction

Page 6: Glycine metabolism & its clinical significance

Synthesis of Glycine from Threonine

Synthesis of Glycine fromThreonine

Page 7: Glycine metabolism & its clinical significance

Synthesis of Glycinefrom

Glyoxalate

Page 8: Glycine metabolism & its clinical significance

Degradation of Glycine

Page 9: Glycine metabolism & its clinical significance

Degradation of Glycine to Ammonia &CO2

Role of THF in Glycine Metabolism and Ammonia synthesis

Page 10: Glycine metabolism & its clinical significance

Degradation of Glycine to Ammonia &CO2

Role of THF in Glycine Metabolism

Page 11: Glycine metabolism & its clinical significance

Role of Glycine in synthesis of Cysteine

Page 12: Glycine metabolism & its clinical significance

Role of Glycine biosynthesis of molecules

Page 13: Glycine metabolism & its clinical significance

Glycine links many Metabolic pathways & biomolecules

Page 14: Glycine metabolism & its clinical significance

Functions of Glycine: Collagen synthesis1. Collagen –rich in Glycine -30%

Page 15: Glycine metabolism & its clinical significance

Functions of Glycine :Formation of purine ring2.Formation of purine ring : N7

Page 16: Glycine metabolism & its clinical significance

Functions of Glycine -3.Synthesis of Glutathione

Page 17: Glycine metabolism & its clinical significance

Functions of Glycine in Detoxification by conjugation reaction

4.Detoxification by conjugation reaction

• Bile acids cholic acid /cheno cholic acid

• Cholic acid + Glycine Glycocholic acid

• Chenodeoxy cholic acid+ Glycine Glyco- cheno deoxy cholic acid

• Benzoic acid +Glycine Hippuric acid (excreted by kidney )

Page 18: Glycine metabolism & its clinical significance

Functions of Glycine in synthesis of Heme

5.synthesis of Heme

Succinyl CoA + Glycine δ Amino levulinate (ALA )

HEME

ALA SYNTHTASE

Page 19: Glycine metabolism & its clinical significance

Synthesis of Heme from Glycine

Page 20: Glycine metabolism & its clinical significance

Functions of Glycine: Biosynthesis of Creatine

Source of EnergyIn muscles

Excretion in urine

Page 21: Glycine metabolism & its clinical significance

Creatine & creatinine : clinical significance

Creatine Creatinine

Serum 0.2 -0.6 mg/ dl 0.6 -1.0 mg/ dl

Urine 0 – 50 mg/day 1 -2 gm /day

Creatinine estimation–KFT ( CREATININE –ENDOGENOUS ,NOT ABSORBED NOR SECRETED SECRETION,DAILY EXCRETION )

Creatinine coefficient - mg / kg body weight / dayMan - 24-26 mg / kg body weight / dayWoman - 20- 22 mg / kg body weight / day

Creatnuria : muscular dystrophy /Diabetes Mellitus / Hyperthyroidism/ starvation

Page 22: Glycine metabolism & its clinical significance

Arginine ,Glycine & Methionine (SAM)involved insynthesis of Creatine

Page 23: Glycine metabolism & its clinical significance

Metabolic disorders of GlycineGlycinuria

• Serum Glycine concentration is normal/increased in excretion of Glycine in urine ( defective renal reabsorption of Glycine )

• Increased formation of urinary oxlate calculi (stones )

Primary hyperoxaluria

Increased formation of urinary oxlate calculi

oxalosis ( deposition in various tissue )

Generally defective Glycine transaminase

+ impairment in Glyoxalate oxidation to Formate

Vitamin B12 deficiency :Increased formation of urinary oxlate calculi(stones ) corrected by Vitamin B12 supplementation

Page 24: Glycine metabolism & its clinical significance

Glycinuria :Metabolic disorders of Glycine

Page 25: Glycine metabolism & its clinical significance

Role of Glycine in oxalate formation

Page 26: Glycine metabolism & its clinical significance

Primary Oxaluria type Ileads to renal /kidney stone formation & is responsible for mental retardation

Page 27: Glycine metabolism & its clinical significance

Kidney stones ( Calcium oxalate )

Page 28: Glycine metabolism & its clinical significance

Hepatocytes of liver are involved inOxalate synthesis & renal cells are Involved in oxalate excretion

Page 29: Glycine metabolism & its clinical significance

Summary Of Metabolic pathways of Glycine

Page 30: Glycine metabolism & its clinical significance