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Succinate-CoA Ligase, ADP-Forming, beta Subunit Proteins (SUCLA2)

Succinyl-CoA synthetase (SCS) is a mitochondrial matrix enzyme that acts as a heterodimer, being composed of an invariant alpha subunit and a substrate-specific beta subunit. Additionally we are shipping SUCLA2 Antibodies (14) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
SUCLA2 20916 Q9Z2I9
SUCLA2 8803 Q9P2R7
SUCLA2 511090 Q148D5
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Top SUCLA2 Proteins at antibodies-online.com

Showing 8 out of 8 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
Yeast Mesocricetus auratus His tag   1 mg Log in to see 56 to 66 Days
$2,552.00
Details
Yeast Macaca fascicularis His tag   1 mg Log in to see 56 to 66 Days
$3,142.33
Details
Yeast Cow His tag   1 mg Log in to see 56 to 66 Days
$3,142.33
Details
Yeast Wild boar His tag   1 mg Log in to see 56 to 66 Days
$3,142.33
Details
HOST_Human Human Un-conjugated   20 μg Log in to see 9 to 11 Days
$785.40
Details

SUCLA2 Proteins by Origin and Source

Origin Expressed in Conjugate
Mouse (Murine)

Human , ,
,
Cow (Bovine)

More Proteins for Succinate-CoA Ligase, ADP-Forming, beta Subunit (SUCLA2) Interaction Partners

Mouse (Murine) Succinate-CoA Ligase, ADP-Forming, beta Subunit (SUCLA2) interaction partners

  1. Sucla2(SAbetageo) homozygotes exhibited recessive lethality, with most mutants dying late in gestation (e18.5). Mutant placenta and embryonic (e17.5) brain, heart and muscle showed varying degrees of mitochondrial DNA depletion.

  2. ADP and GDP succinyl-CoA (show OXCT1 Proteins) synthetases have different expression patterns and specificity

Human Succinate-CoA Ligase, ADP-Forming, beta Subunit (SUCLA2) interaction partners

  1. The absence of SUCLA2 and SUCLG2 (show SUCLG2 Proteins) in human glia is in compliance with the presence of alternative pathways occurring in these cells, namely the GABA shunt and ketone body metabolism

  2. SUCLA2 mutations should be analyzed in patients with slowly progressive encephalomyopathy, even in the absence of methylmalonic aciduria or mitochondrial DNA depletion.

  3. This study demonstrated that SUCLA2 is expressed exclusively in neurons in the human cerebral cortex.

  4. Identification of a novel mutation in SUCLA2 in two cousins affected with encephalomyopathy, is reported.

  5. A novel homozygous mutation in SUCLA2 gene has been associated with severe mitochondrial encephalomyopathies in two Italian siblings.

  6. 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 (show SUCLG1 Proteins)).

  7. X-linked sideroblastic anemia due to carboxyl-terminal ALAS2 (show ALAS2 Proteins) mutations that cause loss of binding to the beta-subunit (show POLG Proteins) of succinyl-CoA (show OXCT1 Proteins) synthetase (SUCLA2).

  8. Mitochondrial DNA depletion, encephalomyopathic form, with methylmalonic aciduria is associated with mutations in SUCLA2, the gene encoding a beta subunit of succinate-CoA ligase.

  9. Deficiency of SUCLA2 is associated with encephalomyopathy and mitochondrial DNA depletion.

SUCLA2 Protein Profile

Protein Summary

Succinyl-CoA synthetase (SCS) is a mitochondrial matrix enzyme that acts as a heterodimer, being composed of an invariant alpha subunit and a substrate-specific beta subunit. The protein encoded by this gene is an ATP-specific SCS beta subunit that dimerizes with the SCS alpha subunit to form SCS-A, an essential component of the tricarboxylic acid cycle. SCS-A hydrolyzes ATP to convert succinate to succinyl-CoA. Defects in this gene are a cause of myopathic mitochondrial DNA depletion syndrome. A pseudogene of this gene has been found on chromosome 6.

Gene names and symbols associated with SUCLA2

  • succinate-Coenzyme A ligase, ADP-forming, beta subunit (Sucla2)
  • succinate-CoA ligase, ADP-forming, beta subunit (SUCLA2)
  • 4930547K18Rik protein
  • A-BETA protein
  • MTDPS5 protein
  • SCS protein
  • SCS-betaA protein

Protein level used designations for SUCLA2

ATP-specific succinyl-CoA synthetase subunit beta , SCS-betaA , succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial , succinyl-CoA synthetase beta-A chain , ATP-specific succinyl-CoA synthetase, beta subunit , mitochondrial succinyl-CoA ligase [ADP-forming] subunit beta , renal carcinoma antigen NY-REN-39 , ATP-specific succinyl-CoA synthetase beta , Succinyl-CoA ligase [ADP-forming] beta-chain, mitochondrial

GENE ID SPECIES
20916 Mus musculus
8803 Homo sapiens
511090 Bos taurus
485448 Canis lupus familiaris
399540 Sus scrofa
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