Key features and details
- Rabbit polyclonal to P5CS
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
Product nameAnti-P5CS antibody
See all P5CS primary antibodies
DescriptionRabbit polyclonal to P5CS
Tested applicationsSuitable for: WB, IHC-Pmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat
Recombinant fragment, corresponding to amino acids 10-257 of Human P5CS (BC117240).
- Human fetal kidney, liver and spleen lysates.
FormLyophilized:Add 200ul Steriled Distilled Water.
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 98.88% PBS, 1% BSA
Concentration information loading...
PurityImmunogen affinity purified
Purification notesab111977 is purified by a peptide affinity column.
Our Abpromise guarantee covers the use of ab111977 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/1000. Predicted molecular weight: 87 kDa.|
|IHC-P||1/100 - 1/500.|
PathwayAmino-acid biosynthesis; L-proline biosynthesis; L-glutamate 5-semialdehyde from L-glutamate: step 1/2.
Amino-acid biosynthesis; L-proline biosynthesis; L-glutamate 5-semialdehyde from L-glutamate: step 2/2.
Involvement in diseaseDefects in ALDH18A1 are the cause of mental retardation-joint hypermobility-skin laxity with or without metabolic abnormalities (MRJHSL) [MIM:612652]. Clinical manifestations include microcephaly, progressive neurologic dysfunction, mental retardation, progeroid appearance, joint hypermobility, skin laxity and hyperelasticity, cataracts. Some patients manifest metabolic disturbances such as hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia.
Sequence similaritiesIn the N-terminal section; belongs to the glutamate 5-kinase family.
In the C-terminal section; belongs to the gamma-glutamyl phosphate reductase family.
Cellular localizationMitochondrion inner membrane.
- Information by UniProt
FormP5CS catalyzes the ATP- and NADPH-dependent conversion of L-glutamate to glutamic gamma-semialdehyde, which is the metabolic precursor for proline biosynthesis. There are 2 isoforms produced by alternative splicing.
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ab111977 has been referenced in 1 publication.
- Fischer B et al. Severe congenital cutis laxa with cardiovascular manifestations due to homozygous deletions in ALDH18A1. Mol Genet Metab 112:310-6 (2014). PubMed: 24913064