Recombinant Human BOLA3 protein (His tag) (ab201887)
Key features and details
- Expression system: Escherichia coli
- Purity: > 95% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: SDS-PAGE, MS
Description
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Product name
Recombinant Human BOLA3 protein (His tag) -
Purity
> 95 % SDS-PAGE.
ab201887 was purified using conventional chromatography. -
Expression system
Escherichia coli -
Accession
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Protein length
Full length protein -
Animal free
No -
Nature
Recombinant -
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Species
Human -
Sequence
MGSSHHHHHHSSGLVPRGSHMGSMAAWSPAAAAPLLRGIRGLPLHHRMFA TQTEGELRVTQILKEKFPRATAIKVTDISGGCGAMYEIKIESEEFKEKRT VQQHQMVNQALKEEIKEMHGLRIFTSVPKR -
Predicted molecular weight
15 kDa including tags -
Amino acids
1 to 107 -
Tags
His tag N-Terminus -
Additional sequence information
NCBI Accession No.: NP_997717
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Associated products
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Related Products
Specifications
Our Abpromise guarantee covers the use of ab201887 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
SDS-PAGE
Mass Spectrometry
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Mass spectrometry
MALDI-TOF -
Form
Liquid -
Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
pH: 7.40
Constituents: 89% PBS, 10% Glycerol (glycerin, glycerine)
General Info
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Alternative names
- BolA-like protein 3
- BOLA3
- BOLA3_HUMAN
- MMDS2
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Tissue specificity
Widely expressed. -
Involvement in disease
Defects 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 similarities
Belongs to the bolA/yrbA family. -
Cellular localization
Secreted. Could be secreted via a non-classical export pathway. - Information by UniProt
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab201887 has not yet been referenced specifically in any publications.