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The antibody against BOLA3 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-68 of human BOLA3 (NP_997717.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, ELISA.
The antibody against BOLA3 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-68 of human BOLA3 (NP_997717.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, ELISA.
| Cat.No | ADA-04695A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | BOLA3 |
| Target Synonyms | MMDS2; BOLA3 | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.01% thimerosal, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Mouse heart, Mouse kidney, Mouse stomach, Rat liver | Application | ELISA, WB, IHC-P |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 1-68 of human BOLA3 (NP_997717.2). | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | MAAWSPAAAAPLLRGIRGLPLHHRMFATQTEGELRVTQILKEKFPRATAIKVTDISGGCGAMYEIKIE | Uniprot ID | Q53S33 |
Uniprot Id
Q53S33
Target Species
Human
Target Name
BOLA3
Target Full Name
BolA-like protein 3
Target 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.
Target Involvement
Multiple mitochondrial dysfunctions syndrome 2 with hyperglycinemia (MMDS2)
Target Subcellular Location
Mitochondrion.
Target Protein Families
BolA/IbaG family
Target Tissue Specificity
Widely expressed.
Target Synonyms
BolA-like protein 3; BOLA3; BOLA3_HUMAN; MMDS2
Target Background
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).
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