Key features and details
- Rabbit polyclonal to BOLA3
- Suitable for: IHC-P
- Reacts with: Human
- Isotype: IgG
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 40% Glycerol, 59% PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab185339 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
Tissue specificityWidely expressed.
Involvement in diseaseDefects in BOLA3 are the cause of multiple mitochondrial dysfunctions syndrome type 2 (MMDS2) [MIM:614299]. A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes.
Sequence similaritiesBelongs to the bolA/yrbA family.
Cellular localizationSecreted. Could be secreted via a non-classical export pathway.
- Information by UniProt
- BolA-like protein 3 antibody
- BOLA3 antibody
- BOLA3_HUMAN antibody
- MMDS2 antibody
ab185339 has not yet been referenced specifically in any publications.