Anti-P5CS antibody (ab111977)
Key features and details
- Rabbit polyclonal to P5CS
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Overview
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Product name
Anti-P5CS antibody
See all P5CS primary antibodies -
Description
Rabbit polyclonal to P5CS -
Host species
Rabbit -
Tested applications
Suitable for: WBmore details -
Species reactivity
Reacts with: Human
Predicted to work with: Mouse, Rat -
Immunogen
Recombinant fragment, corresponding to amino acids 10-257 of Human P5CS (BC117240).
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Positive control
- Human fetal kidney, liver and spleen lysates.
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General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
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Properties
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Form
Lyophilized:Add 200ul Steriled Distilled Water. -
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles. -
Storage buffer
pH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 98.88% PBS, 1% BSA -
Concentration information loading...
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Purity
Immunogen affinity purified -
Purification notes
ab111977 is purified by a peptide affinity column. -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
Applications
The Abpromise guarantee
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.
Application | Abreviews | Notes |
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WB |
1/500 - 1/1000. Predicted molecular weight: 87 kDa.
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Notes |
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WB
1/500 - 1/1000. Predicted molecular weight: 87 kDa. |
Target
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Pathway
Amino-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 disease
Defects 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 similarities
In 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 localization
Mitochondrion inner membrane. - Information by UniProt
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Database links
- Entrez Gene: 5832 Human
- Entrez Gene: 56454 Mouse
- Entrez Gene: 361755 Rat
- Omim: 138250 Human
- SwissProt: P54886 Human
- SwissProt: Q9Z110 Mouse
- Unigene: 500645 Human
- Unigene: 233117 Mouse
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Form
P5CS 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. -
Alternative names
- 2810433K04Rik antibody
- AI429789 antibody
- Aldehyde dehydrogenase 18 family member A1 antibody
see all
Images
Datasheets and documents
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SDS download
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Datasheet download
References (1)
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