SKU: WP16594 Categories: ,

AL7A1 rabbit pAb

Price range: $268.00 through $328.00

SKU: WP16594-100
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Product details

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],

Specifications

Size50μL/100μL
TargetAL7A1
ReactivityHuman, Mouse, Rat
ApplicationsWB
MW(Calculated)59kD
MW(Observed)
Host SpeciesRabbit
IsotypeIgG
Conjugate/ModificationUnmodified
Recommended Dilution RatioWB 1:500-2000
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration1 mg/ml
ClonalityPolyclonal
Clone Number
ImmunogenSynthesized peptide derived from human AL7A1 AA range: 229-279
SpecificityThis antibody detects endogenous levels of AL7A1 at Human/Mouse/Rat
Gene NameALDH7A1 ATQ1
Protein NameAL7A1
Other Name
SpeciesHuman
Gene ID501
UniprotP49419,
Species.1Mouse
Gene ID.1110695
Uniprot.1Q9DBF1,
Species.2Rat
Gene ID.2291450
Uniprot.2Q64057
Cellular Localization[Isoform 2]: Cytoplasm, cytosol . Nucleus .; [Isoform 1]: Mitochondrion .
Tissue ExpressionAbundant 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.
Signaling_pathway
Research Areas>>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
FunctionCatalytic 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.,
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