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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 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 24 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Supplier Delivery Price Details
Cow Rabbit Un-conjugated WB 100 μL Log in to see 2 to 3 Days
$289.00
Details
Cow Rabbit Un-conjugated WB 100 μL Log in to see 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 Log in to see 1 to 2 Days
$358.60
Details
Human Rabbit Un-conjugated 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 7 to 9 Days
$514.63
Details
Pig Rabbit Biotin DB, EIA, ID, IF, IP, RIA, WB   1 mL Log in to see 6 to 8 Days
$434.50
Details
Human Rabbit Un-conjugated 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 to 13 Days
$491.63
Details
Human Rabbit Un-conjugated ICC, IHC (p), WB   100 μL Log in to see 7 to 9 Days
$507.83
Details
Bat Rabbit Un-conjugated WB   50 μg Log in to see 7 to 9 Days
$551.83
Details
Cow Rabbit Un-conjugated WB   50 μg Log in to see 7 to 9 Days
$551.83
Details
Human Mouse Un-conjugated ELISA, WB Western Blot analysis of SUCLG1 expression in transfected 293T cell line by SUCLG1 polyclonal antibody (A01).<br><br>Lane1:SUCLG1 transfected lysate(35.047 KDa).<br>Lane2:Non-transfected lysate.<br> 50 μL Log in to see 11 to 12 Days
$305.71
Details

SUCLG1 Antibodies by Reactivity, Application, Clonality and Conjugate

Attributes Applications Host Clonality
Human
,

Rat (Rattus)


Mouse (Murine)


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 (show SUCLA2 Antibodies) and in 10% of SUCLG1 patients.

  4. First Chinese report of succinyl-CoA ligase (show SUCLG2 Antibodies) 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 (show SUCLA2 Antibodies) 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 (show SUCLA2 Antibodies)-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 (show MMD Antibodies) and C4-DC carnitine in urines) and basal ganglia MRI (show C7ORF49 Antibodies) lesions are the hallmarks of SCS (show TWIST1 Antibodies) 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 (show AK3 Antibodies)-specific succinyl-CoA (show OXCT1 Antibodies) 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 (show POLG Antibodies).

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

  3. succinyl-CoA (show OXCT1 Antibodies) synthetase is able to protect GTP (show AK3 Antibodies) 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 (show OXCT1 Antibodies) 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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