Human Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial ELISA Kit | ALDH4A1 elisa kit

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

Cat. Num. AAA762517

• Gene Names: ALDH4A1; P5CD; ALDH4; P5CDh
• Reactivity: Human
• Synonyms: Delta-1-pyrroline-5-carboxylate dehydrogenase; mitochondrial; Human Delta-1-pyrroline-5-carboxylate dehydrogenase; mitochondrial ELISA Kit; ALDH4A1/Delta-1-pyrroline-5-carboxylate dehydrogenase; mitochondrial/P5C dehydrogenase/Aldehyde dehydrogenase family 4 member A1/L-glutamate gamma-semialdehyde dehydrogenase/ALDH4/P5CDH/P5CD/P5CDh/P5CDhL/P5CDhS; ALDH4A1 elisa kit

• Reactivity: Human
• Sequence Length: 563

• Samples: Serum, plasma and other biological fluids.
• Assay Type: Sandwich ELISA, Double Antibody
• Detection Range: 0.156-10ng/ml
• Sensitivity: 0.094ng/ml
• Preparation and Storage: Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Typical Testing Data

Typical Standard Curve

Related Product Information for ALDH4A1 elisa kit

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti-Human ALDH4A1 antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Human ALDH4A1 antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRP-Streptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the Human ALDH4A1 amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of Human ALDH4A1 can be calculated.

NCBI and Uniprot Product Information

• NCBI GI #: 25777736
• NCBI GeneID: 8659
• NCBI Accession #: NP_733844.1
• NCBI GenBank Nucleotide #: NM_170726.2
• Molecular Weight: 56,043 Da
• NCBI Official Full Name: delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial isoform a
• NCBI Official Synonym Full Names: aldehyde dehydrogenase 4 family member A1
• NCBI Official Symbol: ALDH4A1
• NCBI Official Synonym Symbols: P5CD; ALDH4; P5CDh
• NCBI Protein Information: delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
• UniProt Protein Name: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
• UniProt Gene Name: ALDH4A1
• UniProt Synonym Gene Names: ALDH4; P5CDH; P5C dehydrogenase
• UniProt Entry Name: AL4A1_HUMAN

NCBI Description

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]

Uniprot Description

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

Chromosomal Location of Human Ortholog: 1p36

Cellular Component: mitochondrial matrix

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

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

Disease: Hyperprolinemia, Type Ii