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Recombinant Human BolA-like protein 3 (BOLA3)

ACP17285

Number
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High Purity LevelsPrecision and ReliabilityCustomization Options

Specifications


Cat.No ACP17285 Target NameBOLA3
Target SynonymsBolA-like protein 3; BOLA3; BOLA3_HUMAN; MMDS2FormLyophilized powder
Expression SystemCustom Production. Please inquire and provide the desire expression system.Expression Range1-107
Protein LengthFull length proteinPurity>85% (SDS-PAGE)
Storage Buffer5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Immunogen Information


Target SpeciesHumanUniprot IDQ53S33
Background Information
  • 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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