Immunogen
Synthesized peptide derived from human AL7A1 AA range: 229-279
Specificity
This antibody detects endogenous levels of AL7A1 at Human/Mouse/Rat
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% 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
The protein encoded by this gene is a member of subfamily 7 in the aldehyde dehydrogenase gene family. These enzymes are thought to play a major role in the detoxification of aldehydes generated by alcohol metabolism and lipid peroxidation. This particular member has homology to a previously described protein from the green garden pea, the 26g pea turgor protein. It is also involved in lysine catabolism that is known to occur in the mitochondrial matrix. Recent reports show that this protein is found both in the cytosol and the mitochondria, and the two forms likely arise from the use of alternative translation initiation sites. An additional variant encoding a different isoform has also been found for this gene. Mutations in this gene are associated with pyridoxine-dependent epilepsy. Several related pseudogenes have also been identified. [provided by RefSeq, Jan 2011],
Function
catalytic activity:L-2-aminoadipate 6-semialdehyde + NAD(P)(+) + H(2)O = L-2-aminoadipate + NAD(P)H.,disease:Defects in ALDH7A1 are the cause of pyridoxine-dependent epilepsy (PDE) [MIM:266100]. PDE is characterized by a combination of various seizure types. It usually occurs in the first hours of life and is unresponsive to standard anticonvulsants, responding only to immediate administration of pyridoxine hydrochloride.,similarity:Belongs to the aldehyde dehydrogenase family.,subunit:Homotetramer.,tissue specificity:Abundant in hepatoma cells and fetal cochlea, ovary, eye, heart, adrenal gland, liver and kidney. Low levels present in adult peripheral blood leukocytes and fetal brain, thymus, spleen, skeletal muscle, lung and tongue.,
Fields
>>Glycolysis / Gluconeogenesis;>>Ascorbate and aldarate metabolism;>>Fatty acid degradation;>>Glycine, serine and threonine metabolism;>>Valine, leucine and isoleucine degradation;>>Lysine degradation;>>Arginine and proline metabolism;>>Histidine metabolism;>>Tryptophan metabolism;>>beta-Alanine metabolism;>>Glycerolipid metabolism;>>Pyruvate metabolism;>>Metabolic pathways;>>Alcoholic liver disease
Human protein sequence Database
P49419
Mouse protein sequence database
Q9DBF1
Rat protein sequence database
Q64057
Cellular localization
[Isoform 2]: Cytoplasm, cytosol . Nucleus .; [Isoform 1]: Mitochondrion .
Tissue expression
Abundant in hepatoma cells and fetal cochlea, ovary, eye, heart, adrenal gland, liver and kidney. Low levels present in adult peripheral blood leukocytes and fetal brain, thymus, spleen, skeletal muscle, lung and tongue.
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