Rabbit Anti-SUCLG1 Recombinant Antibody (CBXS-5440) (CBMAB-S2651-CQ)

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Basic Information

Host Animal
Rabbit
Clone
CBXS-5440
Application
WB, IP, IF
Specificity
Human, Mouse, Rat, Monkey
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
More Infomation

Target

Full Name
Succinate-CoA Ligase Alpha Subunit
Introduction
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.
Entrez Gene ID
Human8802
Mouse56451
Rat114597
Monkey693991
UniProt ID
HumanP53597
MouseQ9WUM5
RatP13086
MonkeyG7NAH3
Alternative Names
GALPHA; MTDPS9; SUCLA1
Function
Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits.
Biological Process
Biological Process succinyl-CoA catabolic process1 PublicationIC:ComplexPortal
Biological Process tricarboxylic acid cycleManual Assertion Based On ExperimentIBA:GO_Central
Cellular Location
Mitochondrion
Involvement in disease
Mitochondrial DNA depletion syndrome 9 (MTDPS9):
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.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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