ALDH4A1 sirna
ALDH4A1 siRNA (Human)
NCBI and Uniprot Product Information
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: Mitochondrial; Oxidoreductase; Amino Acid Metabolism - alanine, aspartate and glutamate; EC 1.2.1.88; Amino Acid Metabolism - arginine and proline
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
Research Articles on ALDH4A1
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Product Notes
The ALDH4A1 aldh4a1 (Catalog #AAA8220559) is a siRNA produced from Synthetic and is intended for research purposes only. The product is available for immediate purchase. The ALDH4A1 siRNA (Human) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's ALDH4A1 can be used in a range of immunoassay formats including, but not limited to, RNA Interference (RNAi). Researchers should empirically determine the suitability of the ALDH4A1 aldh4a1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ALDH4A1, siRNA" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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