-
cysteine by
cystathionine gamma-lyase (CTH). Biosynthetically,
cystathionine is
generated from
homocysteine and
serine by
cystathionine beta synthase...
- the
metabolism of the
amino acid
methionine due to a
deficiency of
cystathionine beta
synthase or
methionine synthase. It is an
inherited autosomal recessive...
- The
enzyme cystathionine γ-lyase (EC 4.4.1.1, CTH or CSE; also cystathionase;
systematic name L-
cystathionine cysteine-lyase (deaminating; 2-oxobutanoate-forming))...
-
Cystathionine-β-synthase, also
known as CBS, is an
enzyme (EC 4.2.1.22) that in
humans is
encoded by the CBS gene. It
catalyzes the
first step of the...
-
serine to
produce cystathionine.
Instead of
degrading cystathionine via
cystathionine-β-lyase, as in the
biosynthetic pathway,
cystathionine is
broken down...
-
homocysteine into
cystathionine. The
cystathionine is then
converted to
hypotaurine by the
sequential action of
three enzymes:
cystathionine gamma-lyase, cysteine...
-
cysteine via homocysteine.
Cystathionine β-synthase
catalyses the
condensation of
homocysteine and
serine to give
cystathionine. This
reaction uses pyridoxine...
- S-adenosylmethionine.
Cystathionine beta-synthase then
combines homocysteine and
serine to form the
asymmetrical thioether cystathionine. The
enzyme cystathionine gamma-lyase...
-
interconversion of
cysteine and
homocysteine through the
intermediate cystathionine. Two
transsulfurylation pathways are known: the
forward and the reverse...
- Stephens, K.; Picker, JD.; Levy, HL. (1993). "Homocystinuria
Caused by
Cystathionine Beta-Synthase Deficiency". PMID 20301697. Viljoen, D. (1994). "Congenital...
