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    SPG3A/Atlastin Polyclonal Antibody, Biotin Conjugated

    Overview
    Catalog # bs-11759R-Biotin
    Product Name SPG3A/Atlastin Polyclonal Antibody, Biotin Conjugated
    Applications WB, IHC-P
    Reactivity Human, Mouse, Rat
    Specifications
    Conjugation Biotin
    Host Rabbit
    Source KLH conjugated synthetic peptide derived from human SPG3A/Atlastin
    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.
    Target
    Synonyms AD FSP; atl1; ATLA1_HUMAN; Atlastin GTPase 1; Atlastin-1; Atlastin1; Brain specic GTP binding protein; Brain-specic GTP-binding protein; FSP1; GBP-3; GBP3; GTP-binding protein 3; Guanine nucleotide-binding protein 3; Guanylate binding protein 3; hGBP3; HSN1D; Spastic paraplegia 3 protein A; SPG 3A; SPG3; SPG3A.
    Background Atlastins are Golgi-localized, integral membrane proteins that function as GTPases. The Atlastin proteins, also designated SPG3A and guanylate-binding protein 3, comprise a Dynamin superfamily that plays a role in axonal maintenance. Hereditary spastic paraplegia (HSP) is an inherited neurodegenerative disorder that is characterized by retrograde axonal degeneration. HSP primarily affects long corticospinal neurons and causes spastic lower extremity weakness. Spastin, a microtubule (MT)-severing AAA ATPase, is a binding partner of Atlastin that is involved in membrane dynamics. This Spastin/Atlastin binding may be involved in the biochemical pathway that leads to HSP development. Mutations in the Atlastin gene (SPG3A) account for approximately 10% of all autosomal dominant HSPs, while mutations in the Spastin gene (SPG4) account for almost 40%.