Anti-SUCLG1 monoclonal antibody (DCABH-201553) Made to order

Rabbit anti-Human SUCLG1 monoclonal antibody for WB, ELISA

View other SUCLG1 antibodies

Specifications


Host Species
Rabbit
Antibody Isotype
IgG
Species Reactivity
Human
Immunogen
A synthetic peptide of human SUCLG1 is used for rabbit immunization.
Conjugate
Unconjugated

Target


Alternative Names
SUCLG1; succinate-CoA ligase, alpha subunit; GALPHA; MTDPS9; SUCLA1; succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
Entrez Gene ID
UniProt ID

Product Background


Gene summary
SUCLG1 (Succinate-CoA Ligase Alpha Subunit) is a Protein Coding gene. Diseases associated with SUCLG1 include mitochondrial dna depletion syndrome 9 and fatal infantile lactic acidosis with methylmalonic aciduria. Among its related pathways are Metabolism and Valproic Acid Pathway, Pharmacodynamics. GO annotations related to this gene include poly(A) RNA binding and GTP binding. An important paralog of this gene is ACLY. 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.
Antigen Description
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. Mitochondrial DNA depletion syndrome 9 (MTDPS9) [MIM:245400]: A severe disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, lactic acidosis, severe psychomotor retardation, progressive neurologic deterioration, and excretion of methylmalonic acid. Note=The disease is caused by mutations affecting the gene represented in this entry. Succinyl-CoA ligase [GDP-forming] subunit alpha, mitochondrial is an enzyme that in humans is encoded by the SUCLG1 gene. The function about SUCLG1 antigen include ATP citrate synthase activity; GDP binding; GTP binding; cofactor binding; poly(A) RNA binding; protein heterodimerization activity; succinate-CoA ligase (ADP-forming) activity; succinate-CoA ligase (GDP-forming) activity.
Pathway
Carbon metabolism; Citrate cycle (TCA cycle); Citrate cycle (TCA cycle, Krebs cycle); Citrate cycle, second carbon oxidation, 2-oxoglutarate oxaloacetate; Citric acid cycle (TCA cycle); Metabolism; Propanoate metabolism; Pyruvate metabolism and Citric Acid (TCA) cycle; TCA cycle; TCA cycle VIII (metazoan); The citric acid (TCA) cycle and respiratory electron transport.

Citations


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We offer labeled antibodies using our catalogue antibody products and a broad range of intensely fluorescent dyes and labels including HRP, biotin, ALP, Alexa Fluor® dyes, DyLight® Fluor dyes, R-phycoerythrin (R-PE), at scales from less than 100 μg up to 1 g of IgG antibody. Learn More

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References


Rizzo, C; Boenzi, S; et al. Measurement of succinyl-carnitine and methylmalonyl-carnitine on dried blood spot by liquid chromatography-tandem mass spectrometry. CLINICA CHIMICA ACTA 429:30-33(2014).
Fellman, V; Kotarsky, H; et al. Mitochondrial hepatopathies in the newborn period. SEMINARS IN FETAL & NEONATAL MEDICINE 16:222-228(2011).

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