Description

  • Product name

    Recombinant Human SPG3A/ATL1 protein
  • Expression system

    Wheat germ
  • Protein length

    Protein fragment
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MAKNRRDRNSWGGFSEKTYEWSSEEEEPVKKAGPVQVLIVKDDHSFELDE TALNRILLSEAVRDKEVVAVSVAGAFRKGKSFLMDFMLRYMYNQESVDWV
    • Amino acids

      1 to 100
    • Tags

      GST tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab153326 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    Western blot

    ELISA

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 mg/ml.

     This product was previously labelled as SPG3A

     

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

    • AD FSP
    • atl1
    • ATLA1_HUMAN
    • Atlastin GTPase 1
    • Atlastin-1
    • Atlastin1
    • Brain specific GTP binding protein
    • Brain-specific 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
    • SPG3A gene
    see all
  • Function

    GTPase tethering membranes through formation of trans-homooligomer and mediating homotypic fusion of endoplasmic reticulum membranes. Functions in endoplasmic reticulum tubular network biogenesis. May also regulate Golgi biogenesis. May regulate axonal development.
  • Tissue specificity

    Expressed predominantly in the adult and fetal central nervous system. Measurable expression in all tissues examined, although expression in adult brain is at least 50-fold higher than in other tissues. Detected predominantly in pyramidal neurons in the cerebral cortex and the hippocampus of the brain. Expressed in upper and lower motor neurons (at protein level).
  • Involvement in disease

    Defects in ATL1 are the cause of spastic paraplegia autosomal dominant type 3 (SPG3) [MIM:182600]; also known as Strumpell-Lorrain syndrome. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs.
  • Sequence similarities

    Belongs to the GBP family. Atlastin subfamily.
  • Cellular localization

    Endoplasmic reticulum membrane. Golgi apparatus membrane. Cell projection > axon.
  • Information by UniProt

Images

  • ab153326 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab153326 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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