ALDH4A1 blocking peptide
ALDH4A1 Blocking Peptide (C-term)
NCBI and Uniprot Product Information
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: 1-pyrroline-5-carboxylate dehydrogenase activity; aldehyde dehydrogenase (NAD) activity; electron carrier activity; identical protein binding
Biological Process: 4-hydroxyproline catabolic process; proline catabolic process; proline metabolic process
Disease: Hyperprolinemia, Type Ii
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Product Notes
The ALDH4A1 aldh4a1 (Catalog #AAA9229371) is a Blocking Peptide and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "ALDH4A1, Blocking Peptide" 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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