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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 and many more products for this protein.

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	Quantity	Delivery	Price	Details
ABIN2790136	Cow	Rabbit	Un-conjugated	WB	100 μL	2 to 3 Days	$289.00	Details
ABIN2790137	Cow	Rabbit	Un-conjugated	WB	100 μL	2 to 3 Days	$289.00	Details
ABIN2856658	Human	Rabbit	Un-conjugated	ICC, IF, IHC (p), WB	100 μL	3 to 4 Days	$466.18	Details
ABIN568429	Pig	Rabbit	Biotin	DB, EIA, ID, IF, IP, RIA, WB	1 mL	6 to 8 Days	$456.50	Details
ABIN1833520	Human	Rabbit	Un-conjugated	ICC, IHC, IHC (p), WB	100 μL	11 to 14 Days	$537.17	Details
ABIN6749437	Cow	Rabbit	Un-conjugated	WB	100 μL	11 to 14 Days	$551.83	Details
ABIN6749436	Bat	Rabbit	Un-conjugated	WB	100 μL	11 to 14 Days	$551.83	Details
ABIN6568655	Human	Rabbit	Un-conjugated	WB	200 μL	12 to 14 Days	$438.90	Details
ABIN1327671	Human	Mouse	Un-conjugated	ELISA, WB	50 μL	11 to 12 Days	$305.71	Details
ABIN797250	Human	Rabbit	Un-conjugated	IP, WB	100 μL	11 to 14 Days	$793.83	Details

More product categories related to Succinate-CoA Ligase, alpha Subunit Antibody

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

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

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.
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
Long survival, to age 20 years or older, was reported in 12% of SUCLA2 and in 10% of SUCLG1 patients.
First Chinese report of succinyl-CoA ligase deficiency caused by novel SUCLG1 mutations; five novel pathogenic mutations in SUCLG1 were identified
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).
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.
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.
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

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

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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