anti-Succinate-CoA Ligase, alpha Subunit (SUCLG1) 抗体

SUCLG1 encodes the alpha subunit of the heterodimeric enzyme succinate coenzyme A ligase. 再加上,我们可以发Succinate-CoA Ligase, alpha Subunit 蛋白 (7)和数多这个蛋白质的别的产品。

列出全部抗体 基因 基因ID UniProt
SUCLG1 8802 P53597
SUCLG1 114597 P13086
SUCLG1 56451 Q9WUM5
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Showing 10 out of 25 products:

产品编号 适用 宿主 标记 应用范围 图像 规格 供应商 交付 价格 详细
Cow 非结合性 WB 100 μL Log in to see 2至3个工作日
$289.00
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Cow 非结合性 WB 100 μL Log in to see 2至3个工作日
$289.00
详细
非结合性 ICC, IF, IHC (p), WB ICC/IF Image SUCLG1 antibody detects SUCLG1 protein at mitochondria by confocal immunofluorescent analysis. Sample: HeLa cells were fixed in 2% paraformaldehyde/culture medium at 37℃ for 30 min. Green: SUCLG1 protein stained by SUCLG1 antibody , diluted at 1:500. Blue: Hoechst 33342 staining. [Images captured by Olympus FV10i Confocal Laser Scanning Microscope] IHC-P Image Immunohistochemical analysis of paraffin-embedded N87 xenograft, using SUCLG1, antibody at 1:100 dilution. 100 μL Log in to see 1至2个工作日
$358.60
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非结合性 ICC, IF, IHC, IHC (p), IP, WB Western Blot: SUCLG1 Antibody [NBP1-32728] - Sample (30 ug of whole cell lysate) A: A431 10% SDS PAGE, antibody diluted at 1:1000. Immunocytochemistry/Immunofluorescence: SUCLG1 Antibody [NBP1-32728] - Sample: HeLa cells were fixed in 2% paraformaldehyde/culture medium at 37C for 30 min. Green: SUCLG1 protein stained by SUCLG1 antibody diluted at 1:500. Blue: Hoechst 33342 staining. 100 μL Log in to see 8至11个工作日
$514.63
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非结合性 ICC, IF, IHC, IHC (p), WB Immunocytochemistry/Immunofluorescence: SUCLG1 Antibody  - Immunofluorescent staining of human cell line A-431 shows localization to plasma membrane & mitochondria. Immunohistochemistry-Paraffin: SUCLG1 Antibody  - Staining of human kidney shows high expression. 0.1 mL Log in to see 10至13个工作日
$491.63
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非结合性 IHC, IHC (p), WB Immunohistochemistry-Paraffin: SUCLG1 Antibody  - Staining in human kidney and pancreas tissues using anti-SUCLG1 antibody. Corresponding SUCLG1 RNA-seq data are presented for the same tissues. Immunohistochemistry-Paraffin: SUCLG1 Antibody  - Staining of human kidney shows high expression. 0.1 mL Log in to see 10至13个工作日
$491.63
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Pig Biotin DB, EIA, ID, IF, IP, RIA, WB   1 mL Log in to see 6至8个工作日
$434.50
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非结合性 ICC, IHC, IHC (p), WB   100 μL Log in to see 7至9个工作日
$507.83
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Bat 非结合性 WB   50 μg Log in to see 7至9个工作日
$551.83
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Cow 非结合性 WB   50 μg Log in to see 7至9个工作日
$551.83
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更多抗Succinate-CoA Ligase, alpha Subunit的相互作用对抗体

Human Succinate-CoA Ligase, alpha Subunit (SUCLG1) interaction partners

  1. We report two Tunisian patients belonging to a consanguineous family with mitochondrial encephalomyopathy. Mutational analysis of SUCLG1 gene showed the presence of c.41T > C in exon 1 in a homozygous state. This mutation substitutes a conserved methionine residue to a threonine at position 14 (p.M14T) located at the SUCLG1 protein mitochondrial targeting sequence.

  2. These abnormal phenotypes are rescued upon ectopic expression of wild-type SUCLG1 in the patient's fibroblasts, thus functionally confirming the pathogenic nature of the SUCLG1 VUS identified in this patient and expanding the phenotypic spectrum for SUCLG1 deficiency

  3. Long survival, to age 20 years or older, was reported in 12% of SUCLA2 (显示 SUCLA2 抗体) and in 10% of SUCLG1 patients.

  4. First Chinese report of succinyl-CoA ligase (显示 SUCLG2 抗体) deficiency caused by novel SUCLG1 mutations; five novel pathogenic mutations in SUCLG1 were identified

  5. 3 novel mutations have been identified in patients with the mitochondrial DNA depletion syndrome (c.1048G>A and c.1049G>T in SUCLA2 (显示 SUCLA2 抗体) and c.531+4A>T in SUCLG1).

  6. Our results suggest that SUCLG1 mutations that lead to complete absence of SUCLG1 protein are responsible for a very severe disorder with antenatal manifestations, whereas a SUCLA2 (显示 SUCLA2 抗体)-like phenotype is found in patients with residual SUCLG1 protein.

  7. This report enlarges the phenotypic spectrum of SUCLG1 mutations and confirms that a characteristic metabolic profile (presence of MMA (显示 MMD 抗体) and C4-DC carnitine in urines) and basal ganglia MRI (显示 C7ORF49 抗体) lesions are the hallmarks of SCS (显示 TWIST1 抗体) defects.

  8. A novel mutation in the SUCLG1 gene was found in two newborns having lethal lactic acidosis, multi-organ failure and congenital malformations including interrupted aortic arch.

Pig (Porcine) Succinate-CoA Ligase, alpha Subunit (SUCLG1) interaction partners

  1. Pig GTP (显示 AK3 抗体)-specific succinyl-CoA (显示 OXCT1 抗体) synthetase was crystallized in the presence of succinate, magnesium ions and CoA, and the structure of the complex was determined by X-ray crystallography to 2.2 A resolution. Succinate binds in the carboxy-terminal domain of the beta-subunit (显示 POLG 抗体).

  2. Structure of GTP-specific succinyl-CoA synthetase subunits in complex with CoA has been resolved.

  3. succinyl-CoA (显示 OXCT1 抗体) synthetase is able to protect GTP (显示 AK3 抗体) from hydrolysis when the active-site histidine residue is not in position to be phosphorylated

Mouse (Murine) Succinate-CoA Ligase, alpha Subunit (SUCLG1) interaction partners

  1. ADP and GDP succinyl-CoA (显示 OXCT1 抗体) synthetases have different expression patterns and specificity

Succinate-CoA Ligase, alpha Subunit (SUCLG1) 抗原简介

蛋白简介

This gene encodes the alpha subunit of the heterodimeric enzyme succinate coenzyme A ligase. This enzyme is targeted to the mitochondria and catalyzes the conversion of succinyl CoA and ADP or GDP to succinate and ATP or GTP. Mutations in this gene are the cause of the metabolic disorder fatal infantile lactic acidosis and mitochondrial DNA depletion.

Gene names and symbols associated with SUCLG1

  • succinate-CoA ligase alpha subunit (SUCLG1) 抗体
  • succinate-CoA ligase, alpha subunit L homeolog (suclg1.L) 抗体
  • succinate-CoA ligase, alpha subunit (suclg1) 抗体
  • succinate-CoA ligase, alpha subunit (Suclg1) 抗体
  • succinate-CoA ligase, GDP-forming, alpha subunit (Suclg1) 抗体
  • 1500000I01Rik 抗体
  • GALPHA 抗体
  • MTDPS9 抗体
  • SCS 抗体
  • Sucla1 抗体
  • zgc:92738 抗体
  • zgc:103729 抗体

Protein level used designations for SUCLG1

succinate-CoA ligase, GDP-forming, alpha subunit , succinate-CoA ligase, GDP-forming alpha subunit , SCS-alpha , succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial , succinyl-CoA ligase [GDP-forming] subunit alpha, mitochondrial , succinyl-CoA synthetase subunit alpha , succinyl-CoA synthetase alpha subunit , SUCLGa

GENE ID SPECIES
470417 Pan troglodytes
475775 Canis lupus familiaris
494796 Xenopus laevis
594977 Xenopus (Silurana) tropicalis
8802 Homo sapiens
399539 Sus scrofa
114597 Rattus norvegicus
509983 Bos taurus
436850 Danio rerio
56451 Mus musculus
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