GeneBio Systems
Recombinant ALDH4A1 Monoclonal Antibody
Recombinant ALDH4A1 Monoclonal Antibody
SKU:AN300262P
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Storage:This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free. Avoid repeated freeze-thaw cycles.
Shipping:Ice bag
Exp date:12 months
Category ID_II:Primary Antibodies
Category ID_III:Recombinant Antibodies
Abbreviation:ALDH4A1
Target Synonym:ALDH;ALDH4A;ALDH4;P5CDH;ALDH4A1;AL4A1;Aldehyde dehydrogenase;aldehyde dehydrogenase 4;aldehyde dehydrogenase 4 family;Aldehyde dehydrogenase family 4 member A1;Delta 1 pyrroline 5 carboxylate dehydrogenase;Delta-1-pyrroline-5-carboxylate dehydrogenase;family 4;L-glutamate gamma-semialdehyde dehydrogenase;member 1;member A1;mitochondrial;mitochondrial delta-1-pyrroline 5-carboxylate dehydrogenase;P5C dehydrogenase;P5CD;P5CDhL;P5CDhS;Pyrroline-5-carboxylate dehydrogenase;RP11 128M10.1;subfamily A
Research Areas:Tags & Cell Markers;Signal Transduction;Metabolism
Conjugation:Unconjugated
Host:Rabbit
Species reactivity:Human
Application:WB;IP
Isotype:IgG
Clonality:Monoclonal
Clone NO.:11B3
UNIProt ID:P30038
Accession:
Background:ALDH4A1 is a member of the aldehyde dehydrogenase family. Aldehyde dehydrogenase enzymes function in the metabolism of many molecules including certain fats (cholesterol and other fatty acids) and protein building blocks (amino acids). Additional aldehyde dehydrogenase enzymes detoxify external substances, such as alcohol and pollutants, and internal substances, such as toxins that are formed within cells. ALDH4A1 is expressed abundantly in liver followed by skeletal muscle, kidney, heart, brain, placenta, lung and pancreas. It is a mitochondrial matrix NAD-dependent dehydrogenase which catalyzes the second step of the proline degradation pathway, converting pyrroline-5-carboxylate to glutamate. 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.
Concentration:1 mg/mL
Immunogen:Recombinant Human ALDH4A1 Protein
Buffer:0.2 μm filtered solution in PBS
Purification method:Protein A
Dilution:WB 1:500-1:2000;IP 1-4 μL/mg of lysate
Calculated MW:62 kDa
ObservedMW:62 kDa
