Anti-P5CS抗体(ab111977)
Key features and details
- Rabbit polyclonal to P5CS
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
概述
-
产品名称
Anti-P5CS抗体
参阅全部 P5CS 一抗 -
描述
兔多克隆抗体to P5CS -
宿主
Rabbit -
经测试应用
适用于: WBmore details -
种属反应性
与反应: Human
预测可用于: Mouse, Rat -
免疫原
Recombinant fragment, corresponding to amino acids 10-257 of Human P5CS (BC117240).
-
阳性对照
- Human fetal kidney, liver and spleen lysates.
-
常规说明
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.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
-
形式
Lyophilized:Add 200ul Steriled Distilled Water. -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles. -
存储溶液
pH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 98.88% PBS, 1% BSA -
Concentration information loading...
-
纯度
Immunogen affinity purified -
纯化说明
ab111977 is purified by a peptide affinity column. -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
-
Compatible Secondaries
-
Isotype control
-
Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab111977于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
---|---|---|
WB |
1/500 - 1/1000. Predicted molecular weight: 87 kDa.
|
说明 |
---|
WB
1/500 - 1/1000. Predicted molecular weight: 87 kDa. |
靶标
-
通路
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. -
疾病相关
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. -
序列相似性
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. -
细胞定位
Mitochondrion inner membrane. - Information by UniProt
-
数据库链接
- 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
-
形式
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. -
别名
- 2810433K04Rik antibody
- AI429789 antibody
- Aldehyde dehydrogenase 18 family member A1 antibody
see all
图片
数据表及文件
-
SDS download
-
Datasheet download
文献 (1)
ab111977 被引用在 1 文献中.
- 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