- In
organic chemistry, keto
acids or
ketoacids (also
called oxo
acids or oxoacids) are
organic compounds that
contain a
carboxylic acid
group (−COOH) and...
- The branched-chain α-
ketoacid dehydrogenase complex (BCKDC or
BCKDH complex) is a multi-subunit
complex of
enzymes that is
found on the
mitochondrial inner...
-
acids due to a
deficiency in the
activity of the branched-chain alpha-
ketoacid dehydrogenase (BCKAD) complex. It
particularly affects the
metabolism of...
-
Transamination is a
chemical reaction that
transfers an
amino group to a
ketoacid to form new
amino acids.This
pathway is
responsible for the deamination...
- to an α-
ketoacid,
typically catalysed by a transaminase. The
reaction can be
generalised as such: R1-amino acid + R2-α-
ketoacid ⇌ R1-α-
ketoacid + R2-amino...
- The α-
Ketoacid-Hydroxylamine (KAHA) Amide-Forming
Ligation is a
chemical reaction that is used to join two
unprotected fragments in
peptide synthesis....
-
transfer the
amino group from
aspartate or
glutamate to the
corresponding ketoacid. In the process, the
cofactor shuttles between PLP and the pyridoxamine...
- use
include kinase, BCK, BCKD kinase,
BCODH kinase, branched-chain alpha-
ketoacid dehydrogenase kinase, branched-chain 2-oxo acid
dehydrogenase kinase, branched-chain...
-
their development.
Meguid discovered mutations in the gene
Branched Chain Ketoacid Dehydrogenase Kinase (BCKDK).
Mutations found in
BCKDK were
found in consanguineous...
- valine, and leucine:
threonine dehydrogenase,
acetohydroxyacid synthase,
ketoacid reductoisomerase,
dihydroxyacid dehydrogenase and aminotransferase. Threonine...
