SULPHUR AMINO ACIDS

• Methionine- Essential
• Cysteine- Non essential

• Homocysteine- Non standard amino acid

• Cystine- Two cysteine usually combine to form cystine
in the protein.

• Dietary cysteine can spare the requirement of

methionine.
Cysteine Methionine

Cystine Homocysteine
 METHIONINE
• Essesntial amino acid.
• First AA in protein biosynthesis.(AUG)
• Degraded to succinyl CoA – Glucogenic.
• On adenylation converted to its active form SAM
 SAM ( S-adenosylmethionine)
• Its also called as active methionine
• Its methyl group donor in various biochemical
reactions. (Transmethylation reactions)
Role of SAM
1. Transmethylation reactions
2. Polyamine synthesis
3. Help in DNA methylation
 CYSTEINE
• Non essential amino acid (from Methionine)
• Glucogenic
• Presence of mercapto group (-SH)
• In protein one cysteine joined with another cysteine
by di sulfide bridge to form cystine
 Synthesis of cysteine
• Form carbon skeleton of serine and sulphur of
methionine.
• Sulphur is transfered from homocysteine by
transsulphuration reaction.
• PLP act as a co enzyme
Degradation of cysteine
 Important products from Cysteine
Taurine
• Non standard amino acid produced from cysteine.
• Cojugate with bile acids and enhance flow of bile and
cholesterol clearance.
• Also it regulate intracelular calcium movement, act as
a osmoregulator and inhibitory neuro transmitter.
• Taurine + cholic acid Taurocholic acid
• Taurine + chenodeoxy cholic acid Taurochenodeoxy
cholic acid
Glutathione (Y glutamyl cysteinyl glycine)
Coenzyme A
• Active form of vitamin B5
PAPS (3’-phosphoadenosine-5-phosphosulphate)
• Sulphite produced in cysteine metabolism oxidised to
suphate and excreted in urine.
• Some sulphate condense with ATP to produce PAPS
• It act as a sulphate group donor in various biochemical
reactions. (sulphation of GAGs)
• Also used for conjugation in detoxification process.
Cystine
 Inborn errors of metabolism
Cystinuria
• Characterised by excretion of cysteine and basic AA
• Defect in the transporter in renal tubules
• More frequent IEM- (1 in 7000)
Complications- At acidic pH low solubility of
cysteine causes renal stones in kidney and urinary
tracts.
Treatment- intakae of large amount of water
• Alkalinising urine by sod. Bicarbonate
• Restriction of dietary cysteine intake
• Drugs like D-pencillamine & captopril.
Cystinosis
• Generalised aminoaciduria
• Cysteine accumulate in lysosomes of kidney, liver,
spleen etc.
• Abnormality in transport across lysosomal membrane.
• Leads to renal failure.
Cystathionuria
• Due to def of cystathionase.
• Mental retardation, endocrine disorder and anemia
• Treatment with B6, folic acid. B12 and diet low in
methionine and rich in cysteine.
 Homocystinuria type-I
• Defect in enz Cystathionine synthase
• Accumulation of homocysteine and methionine
• Thrombosis, osteoporosis and mental retardation are
seen
• Excess homocystiene activates Hagemans factor
leading to platlet aggregation so thrombosis, MI and
pulmonary embolism.
• Some respond to vit B6
• It is managed by diet low in methionine and high in
cysteine.