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

Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial; P5C dehydrogenase; Aldehyde dehydrogenase family 4 member A1; Aldh4a1; 1.2.1.88; L-glutamate gamma-semialdehyde

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

For long term storage, please store the entire kit at -20 degree C.

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Small volumes of Aldh4a1 elisa kit 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.

MBS2884996 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial (Aldh4a1) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing Aldh4a1. The ELISA analytical biochemical technique of the MBS2884996 kit is based on Aldh4a1 antibody-Aldh4a1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect Aldh4a1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, Aldh4a1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

61,841 Da

aldehyde dehydrogenase 4 family, member A1

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

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

P5C dehydrogenase  [Similar Products]

AL4A1_MOUSE

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; Amino Acid Metabolism - alanine, aspartate and glutamate; EC 1.2.1.88; Mitochondrial; Amino Acid Metabolism - arginine and proline

Cellular Component: mitochondrion

Molecular Function: 1-pyrroline-5-carboxylate dehydrogenase activity; aldehyde dehydrogenase (NAD) activity; identical protein binding; oxidoreductase activity; oxidoreductase activity, acting on the aldehyde or oxo group of donors, NAD or NADP as acceptor

Biological Process: glutamate biosynthetic process; metabolic process; proline biosynthetic process; proline metabolic process

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