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BOLA3

This gene encodes a protein that plays an essential role in the production of iron-sulfur (Fe-S) clusters for the normal maturation of lipoate-containing 2-oxoacid dehydrogenases, and for the assembly of the mitochondrial respiratory chain complexes. Mutation in this gene has been associated with multiple mitochondrial dysfunctions syndrome-2. Two alternatively spliced transcript variants encoding different isoforms with distinct subcellular localization have been reported for this gene (PMID:21944046).

Full Name

BolA Family Member 3

Function

Acts as a mitochondrial iron-sulfur (Fe-S) cluster assembly factor that facilitates (Fe-S) cluster insertion into a subset of mitochondrial proteins. Probably acts together with NFU1 (PubMed:27532772).

Cellular Location

Mitochondrion

Involvement in disease

Multiple mitochondrial dysfunctions syndrome 2 with hyperglycinemia (MMDS2): 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.

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Anti-BOLA3 antibodies

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Mouse Anti-BOLA3 Recombinant Antibody (1D12) (CBMAB-A0848-LY)

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Target: BOLA3

Host: Mouse

Antibody Isotype: IgG2a, κ

Specificity: Human

Clone: 1D12

Application*: WB, E

Mouse Anti-BOLA3 Recombinant Antibody (0165) (CBMAB-0165-YC)

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Target: BOLA3

Host: Mouse

Antibody Isotype: IgG1

Specificity: Human

Clone: 0165

Application*: IP, FC

Cattle BOLA3 ELISA Kit (V2LY-0626-LY2330)

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Target: BOLA3

Sensitivity: 0.0041 ng/mL

Detection Range: 0.007-1.5 ng/mL

Sample Type: Serum, Plasma, cell culture supernates

Specificity: Cattle

Assay Type: Sandwich

Reactivity: Cattle

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(P): Predicted

* Abbreviations

AActivation
AGAgonist
APApoptosis
BBlocking
BABioassay
BIBioimaging
CImmunohistochemistry-Frozen Sections
CIChromatin Immunoprecipitation
CTCytotoxicity
CSCostimulation
DDepletion
DBDot Blot

EELISA
ECELISA(Cap)
EDELISA(Det)
ESELISpot
EMElectron Microscopy
FFlow Cytometry
FNFunction Assay
GSGel Supershift
IInhibition
IAEnzyme Immunoassay
ICImmunocytochemistry
IDImmunodiffusion
IEImmunoelectrophoresis

IFImmunofluorescence
IGImmunochromatography
IHImmunohistochemistry
IMImmunomicroscopy
IOImmunoassay
IPImmunoprecipitation
ISIntracellular Staining for Flow Cytometry
LALuminex Assay
LFLateral Flow Immunoassay
MMicroarray
MCMass Cytometry/CyTOF
MDMeDIP

MSElectrophoretic Mobility Shift Assay
NNeutralization
PImmunohistologyp-Paraffin Sections
PAPeptide Array
PEPeptide ELISA
PLProximity Ligation Assay
RRadioimmunoassay
SStimulation
SESandwich ELISA
SHIn situ hybridization
TCTissue Culture
WBWestern Blot

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