ALDH4A1, Polyclonal Antibody

Anti -ALDH4A1, CT (Aldehyde Dehydrogenase Family 4 Member A1, Delta-1-pyrroline-5-carboxylate Dehydrogenase, Mitochondrial, P5C Dehydrogenase, ALDH4, P5CDH)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 1; NC_000001.10 (19197924..19229293, complement). Location: 1p36

Polyclonal

IgG

Rabbit

Recognizes human ALDH4A1.

Purified
Purified by ammonium sulfate precipitation.

Supplied as a liquid in PBS, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-ALDH4A1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

ALDH4A1 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.

Antibodies; Abs to Enzymes, Dehydrogenase

ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)

Suitable for use in ELISA, Western Blot, and Immunohistochemistry.
Dilution: ELISA: 1:1,000
Western Blot: 1:50-1:100
Immunohistochemistry: 1:50-1:100

61,719 Da[Similar Products]

aldehyde dehydrogenase 4 family, member A1

delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial; P5C dehydrogenase; OTTHUMP00000002544; OTTHUMP00000002545; aldehyde dehydrogenase family 4 member A1; mitochondrial delta-1-pyrroline 5-carboxylate dehydrogenase

Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial

ALDH4; P5CDH  [Similar Products]

AL4A1_HUMAN

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]

ALDH4A1: 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. Defects in ALDH4A1 are the cause of hyperprolinemia type 2 (HP-2). HP-2 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. Belongs to the aldehyde dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Oxidoreductase; EC 1.2.1.88; Amino Acid Metabolism - arginine and proline; Amino Acid Metabolism - alanine, aspartate and glutamate; Mitochondrial

Chromosomal Location of Human Ortholog: 1p36

Cellular Component: mitochondrial matrix

Molecular Function: identical protein binding; aldehyde dehydrogenase (NAD) activity; electron carrier activity; 1-pyrroline-5-carboxylate dehydrogenase activity

Biological Process: 4-hydroxyproline catabolic process; proline catabolic process; proline biosynthetic process; glutamate biosynthetic process; proline catabolic process to glutamate; proline metabolic process

Disease: Hyperprolinemia, Type Ii

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