{"product_id":"al4a1-rabbit-polyclonal-antibody-es18407","title":"AL4A1 Rabbit Polyclonal Antibody - ES18407","description":"\u003cp\u003eAL4A1 Rabbit Polyclonal Antibody\u003c\/p\u003e\n\n\u003cp\u003eSizes: 50μL, 100μL\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: ES18407-50, ES18407-100\u003c\/p\u003e\n\n\u003cp\u003eLead times: 1-2 weeks, if manufacturer has product in stock\u003c\/p\u003e\n\n\u003cp\u003eManufacturer\/Ship Location: China\u003c\/p\u003e\n\n\u003cp\u003eBackground: 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],\u003c\/p\u003e\n\n\u003cp\u003eReactivity: Human, Mouse, Rat\u003c\/p\u003e\n\n\u003cp\u003eHost: Rabbit\u003c\/p\u003e\n\n\u003cp\u003eApplications: WB\u003c\/p\u003e\n\n\u003cp\u003eAlternate Names: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial (P5C dehydrogenase) (EC 1.5.1.12) (Aldehyde dehydrogenase family 4 member A1)\u003c\/p\u003e\n\n\u003cp\u003eClonality: Polyclonal\u003c\/p\u003e\n\n\u003cp\u003eImmunogen: Synthesized peptide derived from human AL4A1 AA range: 39-89\u003c\/p\u003e\n\n\u003cp\u003eIsotype: IgG\u003c\/p\u003e\n\n\u003cp\u003eTarget: AL4A1\u003c\/p\u003e\n\n\u003cp\u003eSpecificity: This antibody detects endogenous levels of AL4A1 at Human\/Mouse\/Rat\u003c\/p\u003e\n\n\u003cp\u003eDilution: WB 1:500-2000\u003c\/p\u003e\n\n\u003cp\u003ePurification: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.\u003c\/p\u003e\n\n\u003cp\u003eConcentration: 1 mg\/ml,\u003c\/p\u003e\n\n\u003cp\u003eFormlation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.103% sodium azide.\u003c\/p\u003e\n\n\u003cp\u003eExpression: Highest expression is found in liver followed by skeletal muscle, kidney, heart, brain, placenta, lung and pancreas,\u003c\/p\u003e\n\n\u003cp\u003eObserved Band (KD): 60kD\u003c\/p\u003e\n\n\u003cp\u003eFields: Alanine, aspartate and glutamate metabolism, Arginine and proline metabolism, Metabolic pathways\u003c\/p\u003e\n\n\u003cp\u003eFunction: Catalytic Activity: (S)-1-pyrroline-5-carboxylate + NAD(P)(+) + 2 H(2)O = L-glutamate + NAD(P)H\u003cbr\u003e\nDisease: 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\u003cbr\u003e\nFunction: 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\u003cbr\u003e\nPathway: Amino-acid degradation, L-proline degradation into L-glutamate, L-glutamate from L-proline: step 2\/2\u003cbr\u003e\nSimilarity: Belongs to the aldehyde dehydrogena\u003c\/p\u003e\n\n\u003cp\u003eSubcellular Location: Mitochondrion matrix\u003c\/p\u003e\n\n\u003cp\u003eStorage and Stability: -20°C\/1 year\u003c\/p\u003e\n\n\u003cp\u003eGene Name: ALDH4A1 ALDH4 P5CDH\u003c\/p\u003e\n\n\u003cp\u003eProtein Name: AL4A1\u003c\/p\u003e\n\n\u003cp\u003eMouse Gene ID: 212647\u003c\/p\u003e\n\n\u003cp\u003eMouse SwissProt Number: Q8CHT0\u003c\/p\u003e\n\n\u003cp\u003eRat SwissProt Number: P0C2X9\u003c\/p\u003e\n\n\u003cp\u003eResearch Use Only\u003c\/p\u003e","brand":"ELK Biotechnology","offers":[{"title":"50μL","offer_id":46315827626163,"sku":"ES18407-50","price":186.0,"currency_code":"CAD","in_stock":true},{"title":"100μL","offer_id":46315827658931,"sku":"ES18407-100","price":372.0,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/files\/elk_antibodies.jpg?v=1793190497","url":"https:\/\/afsbio.com\/products\/al4a1-rabbit-polyclonal-antibody-es18407","provider":"AFSBio Inc.","version":"1.0","type":"link"}