Molecular weight (DA)
60kD
Immunogen
Synthesized peptide derived from human AL4A1 AA range: 39-89
Specificity
This antibody detects endogenous levels of AL4A1 at Human/Mouse/Rat
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.103% sodium azide.
Dilution rate
WB 1:500-2000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Background
This protein belongs to the aldehyde dehydrogenase family of proteins. This enzyme is a mitochondrial matrix NAD-dependent dehydrogenase which catalyzes the second step of the proline degradation pathway, converting pyrroline-5-carboxylate to glutamate. Deficiency of this enzyme is associated with type II hyperprolinemia, an autosomal recessive disorder characterized by accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jun 2009],
Function
catalytic activity:(S)-1-pyrroline-5-carboxylate + NAD(P)(+) + 2 H(2)O = L-glutamate + NAD(P)H.,disease:Defects in ALDH4A1 are the cause of hyperprolinemia type II (HPII) [MIM:239510]. HPII is characterized by the accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. The disorder may be causally related to neurologic manifestations, including seizures and mental retardation.,function:Irreversible conversion of delta-1-pyrroline-5-carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes.,pathway:Amino-acid degradation; L-proline degradation into L-glutamate; L-glutamate from L-proline: step 2/2.,similarity:Belongs to the aldehyde dehydrogena
Gene Name
ALDH4A1 ALDH4 P5CDH
Other name
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial (P5C dehydrogenase) (EC 1.5.1.12) (Aldehyde dehydrogenase family 4 member A1)
Fields
>>Alanine, aspartate and glutamate metabolism;>>Arginine and proline metabolism;>>Metabolic pathways
Human protein sequence Database
P30038
Mouse protein sequence database
Q8CHT0
Rat protein sequence database
P0C2X9
Cellular localization
Mitochondrion matrix.
Tissue expression
Highest expression is found in liver followed by skeletal muscle, kidney, heart, brain, placenta, lung and pancreas.
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