METHIONINE AND CYSTEINE

KUSUM CHHETRI
11TH BATCH BSc.MLT
UCMS,BHAIRAHAWA
 CONTENT
• INTRODUCTION
• METABOLISM
• PRODUCT
• DISORDER
 INTRODUCTION
• Methionine cysteine and cystine are sulfur
containing glucogenic amino acid.
• Among these methionine is essential amino
acid.
• Methionine serves as a precursor for the
synthesis of cysteine and cystine which are
non essential amino acid.
 METABOLISM
• Activation of methionine and
transmethylation
• Conversion of methionine to cysteine
• Degradation of cystine.
Activation of methionine and
transmethylation
Conversion of methionine to cysteine

Homocysteine Serine

PLP
Cystathionine beta
H₂O sythase

Cystathionine
 Cystathionine
Cystathionase
H₂O PLP

Homoserine Cysteine

NH₃
ᾳ-Ketobutyrate
 ᾳ-Ketobutyrate

NAD⁺
CoA ᾳ-ketobutyrate
dehydrogenase
NADH+H⁺
CO₂

Propionyl CoA
Metabolism of cysteine
 Products derived from cysteine
1. CO-enzyme

decarboxylation
Cysteine Mercaptoethanolamine
Pentothenic
acid

Coenzyme A
 CONT........
2. Tourine
Cysteine
Cysteine deoxygenase

Cysteine sulfinate
oxidization

Cystic acid

Tourine
 Glutathione
• Glutathione is a tripeptide (3 amino acid and 2
peptide bond).
• Glutathione synthesis take place in two steps:-
1. glutamate + cysteine gamma-
glutamyl cysteine
2. glutamyl cysteine +glycine glutathione
both steps need hydrolysis of one ATP each.696
Disorder
• Cystinuria
• Cystinosis
• Homocystinuria
• Cystathioninuria
 Cystinuria
• It is the most common inborn error of amino acid
transport.
• It is autosomal recessive.
• It is due to impaired Reabsorption of
cystine,ornithine, Arginine,Lysine(COAL).
• It is relatively insouble and increased in its
concentration leads to precipitation and formation
of cystine stone in kidney and urinary tract.
• It is identified by cyanide nitroprusside test.
Cytinosis(cystine storage disease)
• It is lysosomal disorder.
• There is an abnormality in the transport of
cystine outside the lysosomes.
• Cystine accumulates in liver, spleen, bone
marrow,kidney,cornea and lymph nodes.
 Forms homocysteine thiolactone →thiolates LDL particles →
aggregate→ endocytosed by macrophges → foam cell→ increase
tendency for atherogenesis

Binds to copper Activation of Hageman’s

↓ factor(XII)
Decrease activity Homocysteine ↓
of lysyl oxidase Increased platelet
↓ adhesiveness
Immature collagen ↓
and elastin Life-threatening intra
↓ vascular thrombosis
Skeletal
Exposure of endothelial cells to homocysteine leads
deformities and
to reduced bioavailability of nitric oxide resulting in
endothelial impaired endothelium-dependent vasodilator
dysfunction function
 Homocystinuria
• Homocystinurias are disorder of methionine
metabolism.
• It is metabolic disorders characterized by the
accumulation and increased urinary excretion
of homocysteine and s-adenosylmethionie.