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Glycine

Glycine is the simplest amino acid and is non-essential. It can be synthesized from carbon dioxide, ammonia, and one carbon units via the glycine synthase reaction or from transamination of glyoxylate and glutamate or alanine. Glycine is mainly utilized through the glycine cleavage system or converted to serine and then to pyruvate in the glucogenic pathway. Defects in glycine metabolism can cause disorders like nonketotic hyperglycinemia or primary hyperoxaluria.

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73 views22 pages

Glycine

Glycine is the simplest amino acid and is non-essential. It can be synthesized from carbon dioxide, ammonia, and one carbon units via the glycine synthase reaction or from transamination of glyoxylate and glutamate or alanine. Glycine is mainly utilized through the glycine cleavage system or converted to serine and then to pyruvate in the glucogenic pathway. Defects in glycine metabolism can cause disorders like nonketotic hyperglycinemia or primary hyperoxaluria.

Uploaded by

Saloni Saloni
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© © All Rights Reserved
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GLYCINE

• It is the simplest amino acid.


• It is non-essential and is glucogenic.
Synthesis of Glycine
From CO2, NH3 :
• Glycine can be synthesized by the glycine synthase reaction
from CO2, NH3 and one carbon unit.
• This is the reversal of the glycine cleavage system. It is a
multienzyme complex.
• It needs the co-enzymes, NAD, lipoamide, tetrahydrofolic acid
and pyridoxal phosphate.

From transamination:
Glycine amino transferase can catalyze the synthesis of glycine
from glyoxylate and glutamate or alanine
Utilization of Glycine
1. Glycine cleavage system
• Glycine undergoes oxidative deamination (reversal of glycine
synthase) to form NH3, CO2 and the one carbon unit
methylene THFA.
• This pathway is the major catabolic route for glycine. The
glycine cleavage system is a multi-enzyme complex consisting
of:
A. Glycine decarboxylase with pyridoxal phosphate
B. Lipoamide containing amino methyl transferase
C. Methylene THFA synthesizing enzyme
D. NAD+ dependent lipoamide dehydrogenase.
2. Glucogenic Pathway
• Glycine is mainly channelled into the glucogenic pathway by
getting first converted to serine.
• This is the reversal of serine hydroxy methyl transferase
reaction.
• The serine is then converted to pyruvate by serine
dehydratase
Glycine may be used for the biosynthesis of the following
compounds and activities
• Creatine, creatine phosphate and creatinine
• Heme
• Purine nucleotides
• Glutathione
• Conjugation of bile acids
• Detoxification of benzoate to form hippurate
• Collagen
• Inhibitory neurotransmitter
• Contributor to one-carbon pool, glycine cleavage system
• Glucogenic
• Conversion to serine
• Acts as a neurotransmitter
In brain stem and spinal cord
At moderate levels, disrupts neuronal traffic
At very high levels causes overexitation

• Is a constituent of protein
Seen at bends or turns
Disorders due to Defective Glycine Metabolism

Non ketotic hyperglycinemia


• Defect in glycine cleavage system
• Glycine level increased in blood, urine & CSF
• Severe mental retardation & seizures
• No effective management
Primary Hyperoxaluria (TYPE -1)
• Due to protein targeting defect
• Glyoxylate amino transferase is seen in mitochondria instead
of in peroxisomes
• Degradation of glyoxylate does not occur
• Increased accumulation of glyoxylate & oxalic acid
• Oxalates deposit in kidney
• Nephrolithiasis, renal colic & hematuria
• Extrarenal oxalosis can occur in heart, blood vessels & bone
Type 2 Hyperoxaluria
• Milder condition
• Deficient activity of cytoplasmic glyoxylate reductase
• Urolithiasis

Management
• Increase water intake  Increase oxalate excretion
• Minimize dietary intake of oxalates
• Restrict intake of leafy vegetables, tea, cocoa, beetroot,
spinach etc.

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