Succinate-CoA Ligase, alpha Subunit Proteine (SUCLG1)

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 viele weitere Produktgruppen zu diesem Protein an.

alle Proteine anzeigen Gen GeneID UniProt
SUCLG1 8802 P53597
SUCLG1 114597 P13086
SUCLG1 56451 Q9WUM5
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Showing 6 out of 7 products:

Katalog Nr. Origin Quelle Konjugat Bilder Menge Anbieter Lieferzeit Preis Details
Insektenzellen Maus His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 50 Days
$5,442.50
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Insektenzellen Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Anmelden zum Anzeigen 50 Days
$5,442.50
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Wheat germ Human GST tag 10 μg Anmelden zum Anzeigen 11 bis 12 Tage
$405.71
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Hefe Rind (Kuh) His tag   1 mg Anmelden zum Anzeigen 60 bis 71 Tage
$2,816.00
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Hefe Wildschwein His tag   1 mg Anmelden zum Anzeigen 60 bis 71 Tage
$2,816.00
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Hefe Ratte His tag   1 mg Anmelden zum Anzeigen 60 bis 71 Tage
$2,816.00
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SUCLG1 Proteine nach Spezies und Herkunft

Origin Exprimiert in Konjugat
Human ,
,
Rat (Rattus)

Mouse (Murine)

Weitere Proteine zu Succinate-CoA Ligase, alpha Subunit (SUCLG1) Interaktionspartnern

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

  1. 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

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

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

  4. 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 Proteine) and c.531+4A>T in SUCLG1).

  5. 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 Proteine)-like phenotype is found in patients with residual SUCLG1 protein.

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

  7. 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 Proteine)-specific succinyl-CoA (zeige OXCT1 Proteine) 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 Proteine).

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

  3. succinyl-CoA (zeige OXCT1 Proteine) synthetase is able to protect GTP (zeige AK3 Proteine) 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 Proteine) synthetases have different expression patterns and specificity

Succinate-CoA Ligase, alpha Subunit (SUCLG1) Protein Überblick

Protein Überblick

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

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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