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

SUCLG1 encodes the alpha subunit of the heterodimeric enzyme succinate coenzyme A ligase. Additionally we are shipping Succinate-CoA Ligase, alpha Subunit Proteins (7) and and many more products for this protein.

list all antibodies Gene Name GeneID UniProt
SUCLG1 8802 P53597
SUCLG1 114597 P13086
SUCLG1 56451 Q9WUM5
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Top anti-Succinate-CoA Ligase, alpha Subunit Antibodies at antibodies-online.com

Showing 10 out of 23 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Delivery Price Details
Cow Rabbit Un-conjugated WB 100 μL 2 to 3 Days
$289.00
Details
Cow Rabbit Un-conjugated WB 100 μL 2 to 3 Days
$289.00
Details
Human Rabbit Un-conjugated 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 3 to 4 Days
$466.18
Details
Pig Rabbit Biotin DB, EIA, ID, IF, IP, RIA, WB   1 mL 6 to 8 Days
$456.50
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Human Rabbit Un-conjugated ICC, IHC, IHC (p), WB   100 μL 11 to 14 Days
$537.17
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Cow Rabbit Un-conjugated WB   100 μL 11 to 14 Days
$551.83
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Bat Rabbit Un-conjugated WB   100 μL 11 to 14 Days
$551.83
Details
Human Rabbit Un-conjugated WB Western blot analysis of extracts of various cell lines, using SUCLG1 antibody. 200 μL 12 to 14 Days
$438.90
Details
Human Mouse Un-conjugated ELISA, WB Western Blot analysis of SUCLG1 expression in transfected 293T cell line by SUCLG1 polyclonal antibody (A01).Lane1:SUCLG1 transfected lysate(35.047 KDa).Lane2:Non-transfected lysate. 50 μL 11 to 12 Days
$305.71
Details
Human Rabbit Un-conjugated IP, WB   100 μL 11 to 14 Days
$793.83
Details

More Antibodies against Succinate-CoA Ligase, alpha Subunit Interaction Partners

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 and in 10% of SUCLG1 patients.

  4. First Chinese report of succinyl-CoA ligase 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 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-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 and C4-DC carnitine in urines) and basal ganglia MRI lesions are the hallmarks of SCS 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-specific succinyl-CoA 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.

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

  3. succinyl-CoA synthetase is able to protect GTP 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 synthetases have different expression patterns and specificity

Succinate-CoA Ligase, alpha Subunit (SUCLG1) Antigen Profile

Protein Summary

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

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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