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    SCA28/AFG3L2 Polyclonal Antibody

    Catalog # bs-11704R
    Product Name SCA28/AFG3L2 Polyclonal Antibody
    Applications WB, IHC-P, IF(IHC-P)
    Reactivity Human, Mouse, Rat
    Conjugation Unconjugated
    Host Rabbit
    Source KLH conjugated synthetic peptide derived from human AFG3L2
    Clonality Polyclonal
    Isotype IgG
    Concentration 1ug/ul
    Purification Purified by Protein A.
    Storage Aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at -20°C for 12 months.
    Synonyms AFG3 ATPase family gene 3, yeast like 2; AFG3 ATPase family gene 3 like 2 yeast; AFG3 ATPase family gene 3 like 2; AFG3 like protein 2; ATPase family gene 3 like 2; ATPase family gene 3 yeast; FLJ25993; Paraplegin like protein; SCA28; Spinocerebellar ataxia 28; AFG32_HUMAN .
    Background AFG3L2 is a multi-pass membrane metalloprotease that contains one AAA (ATPase associated with diverse cellular activities) domain, a zinc-dependent binding motif, an RNA-binding region and an ATP/GTP binding site. Localizing to the mitochondrial membrane, AFG3L2 is ubiquitously expressed with highest expression levels in skeletal muscle and heart. AFG3L2 shares 69% similarity with the yeast Afg3 protein and 49% similarity with Paraplegin, a protein of mitochondria that is thought to be involved in signal transduction and chaperone-like activities. In mitochondria, AFG3L2 forms a complex with Paraplegin that is believed to regulate essential protein quality control. Mutations in the gene encoding either one of these proteins can result in hereditary spastic paraplegia, a degenerative spinal cord disorder that is characterized by muscle spasms, stiffness in the legs and, in some cases, incontinence.