Succinate-CoA Ligase, alpha Subunit (SUCLG1) ELISA Kits

SUCLG1 encodes the alpha subunit of the heterodimeric enzyme succinate coenzyme A ligase. Zusätzlich bieten wir Ihnen Succinate-CoA Ligase, alpha Subunit Antikörper (24) und Succinate-CoA Ligase, alpha Subunit Proteine (7) und viele weitere Produktgruppen zu diesem Protein an.

list all ELISA KIts Gen GeneID UniProt
Anti-Human SUCLG1 SUCLG1 8802 P53597
Anti-Ratte SUCLG1 SUCLG1 114597 P13086
Anti-Maus SUCLG1 SUCLG1 56451 Q9WUM5
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Weitere ELISA Kits für Succinate-CoA Ligase, alpha Subunit Interaktionspartner

Human Succinate-CoA Ligase, alpha Subunit (SUCLG1) Interaktionspartner

  1. Long survival, to age 20 years or older, was reported in 12% of SUCLA2 (zeige SUCLA2 ELISA Kits) and in 10% of SUCLG1 patients.

  2. First Chinese report of succinyl-CoA ligase (zeige SUCLG2 ELISA Kits) deficiency caused by novel SUCLG1 mutations; five novel pathogenic mutations in SUCLG1 were identified

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

  4. 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 (zeige SUCLA2 ELISA Kits)-like phenotype is found in patients with residual SUCLG1 protein.

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

  6. 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) Interaktionspartner

  1. Pig GTP (zeige AK3 ELISA Kits)-specific succinyl-CoA (zeige OXCT1 ELISA Kits) 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 (zeige POLG ELISA Kits).

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

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

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

  1. ADP and GDP succinyl-CoA (zeige OXCT1 ELISA Kits) synthetases have different expression patterns and specificity

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

Beschreibung des Gens

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.

Genbezeichner und Symbole assoziert mit SUCLG1

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

Bezeichner auf Proteinebene für 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 SPEZIES
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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