Atlastin-1 (ATL1), Recombinant Protein

For Research Use Only. Not for use in diagnostic procedures.

Cell Free Expression

Liquid containing glycerol

Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.
Repeated freezing and thawing is not recommended. Store working aliquots at 4 degree C for up to one week.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of ATL1 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Transmembrane Protein

This is a recombinant transmembrane protein expressed in a cell-free expression system.

63,055 Da

atlastin GTPase 1

atlastin-1

Atlastin-1

GBP3; SPG3A; GBP-3; hGBP3  [Similar Products]

ATLA1_HUMAN

The protein encoded by this gene is a GTPase and a Golgi body transmembrane protein. The encoded protein can form a homotetramer and has been shown to interact with spastin and with mitogen-activated protein kinase kinase kinase kinase 4. This protein may be involved in axonal maintenance as evidenced by the fact that defects in this gene are a cause of spastic paraplegia type 3. Three transcript variants encoding two different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

atlastin: 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. Defects in ATL1 are the cause of spastic paraplegia autosomal dominant type 3 (SPG3); 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. Defects in ATL1 are the cause of hereditary sensory neuropathy type 1D (HSN1D). HSN1D is a disease characterized by *****-onset distal axonal sensory neuropathy leading to mutilating ulcerations as well as hyporeflexia. Some patients may show features suggesting upper neuron involvement. Belongs to the GBP family. Atlastin subfamily.

Protein type: Membrane protein, integral; Membrane protein, multi-pass; Vesicle; EC 3.6.5.-

Chromosomal Location of Human Ortholog: 14q22.1

Cellular Component: axon; endoplasmic reticulum; endoplasmic reticulum membrane; Golgi apparatus; Golgi cis cisterna; Golgi membrane; integral to membrane

Molecular Function: GTP binding; GTPase activity; identical protein binding; protein binding

Biological Process: axonogenesis; endoplasmic reticulum organization and biogenesis; metabolic process; protein homooligomerization

Disease: Neuropathy, Hereditary Sensory, Type Id; Spastic Paraplegia 3, Autosomal Dominant

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