P5CS 抗体 (AA 1-224)
Quick Overview for P5CS 抗体 (AA 1-224) (ABIN7599110)
抗原
See all P5CS (ALDH18A1) 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 1-224
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原理
- Anti-P5CS/ALDH18A1 Antibody Picoband®
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交叉反应 （详细）
- No cross-reactivity with other proteins.
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产品特性
- Anti-P5CS/ALDH18A1 Antibody Picoband® (ABIN7599110). Tested in ELISA, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.
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纯化方法
- Immunogen affinity purified.
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免疫原
- E.coli-derived human P5CS/ALDH18A1 recombinant protein (Position: M1-A224).
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亚型
- IgG
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anti-Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1) (AA 696-795) antibody
ALDH18A1 适用: 人 WB, ELISA 宿主: 小鼠 Monoclonal 2B5 unconjugated
anti-Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1) (AA 176-205) antibodyALDH18A1 适用: 人 WB, FACS, IHC (p) 宿主: 兔 Polyclonal RB21608 unconjugated
anti-Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1) (AA 696-795) antibodyALDH18A1 适用: 人 WB, ELISA 宿主: 小鼠 Polyclonal unconjugated
anti-Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1) (AA 1-795) antibodyALDH18A1 适用: 人 WB 宿主: 小鼠 Polyclonal unconjugated
anti-Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1) (AA 35-84) antibodyALDH18A1 适用: 人, 小鼠, 大鼠, Cow, Pig, 豚鼠, 马 WB 宿主: 兔 Polyclonal unconjugated
anti-Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1) (AA 1-240) antibodyALDH18A1 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
anti-Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1) (N-Term) antibodyALDH18A1 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
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应用备注
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Western blot, 0.25-0.5 μg/mL, Human, Mouse, Rat
ELISA, 0.1-0.5 μg/mL, -
1. Bicknell, L. S., Pitt, J., Aftimos, S., Ramadas, R., Maw, M. A., Robertson, S. P. A missense mutation in ALDH18A1, encoding delta-1-pyrroline-5-carboxylate synthase (P5CS), causes an autosomal recessive neurocutaneous syndrome. Europ. J. Hum. Genet. 16: 1176-1186, 2008. 2. Fischer, B., Callewaert, B., Schroter, P., Coucke, P. J., Schlack, C., Ott, C.-E., Morroni, M., Homann, W., Mundlos, S., Morava, E., Ficcadenti, A., Kornak, U. Severe congenital cutis laxa with cardiovascular manifestations due to homozygous deletions in ALDH18A1. Molec. Genet. Metab. 112: 310-316, 2014. 3. Panza, E., Escamilla-Honrubia, J. M., Marco-Marin, C., Gougeard, N., De Michele, G., Brescia Morra, V., Liguori, R., Salviati, L., Donati, M. A., Cusano, R., Pippucci, T., Ravazzolo, R., Nemeth, A. H., Smithson, S., Davies, S., Hurst, J. A., Bordo, D., Rubio, V., Seri, M. ALDH18A1 gene mutations cause dominant spastic paraplegia SPG9: loss of function effect and plausibility of a dominant negative mechanism. (Letter) Brain 139: e3, 2016. Note: Electronic Article. -
限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
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浓度
- 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.01 mg Sodium azide.
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- 4 °C,-20 °C
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储存方法
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Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
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- P5CS (ALDH18A1) (Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1))
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别名
- ALDH18A1
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背景
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Synonyms: Delta-1-pyrroline-5-carboxylate synthase, P5CS, Aldehyde dehydrogenase family 18 member A1, GK, Gamma-glutamyl kinase, GPR, Glutamate-5-semialdehyde dehydrogenase, Glutamyl-gamma-semialdehyde dehydrogenase, ALDH18A1, GSAS, P5CS, PYCS
Tissue Specificity: Detected in pancreas islets (at protein level). Detected in brain and pancreas.
Background: Delta-1-pyrroline-5-carboxylate synthetase (P5CS) is an enzyme that in humans is encoded by the ALDH18A1 gene. This gene is mapped to 10q24.1. This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline, ornithine and arginine. Mutations in this gene lead to hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia and may be associated with neurodegeneration, cataracts and connective tissue diseases. Alternatively spliced transcript variants, encoding different isoforms, have been described for this gene.
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分子量
- 87 kDa
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基因ID
- 5832
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UniProt
- P54886
抗原
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