Atlastin-1, also known as Spastic paraplegia 3 protein A, Guanine nucleotide-binding protein 3, GTP-binding protein 3, GBP3, ATL1 and SPG3A, is a multi-pass membrane protein which belongs to the GBP family and atlastin subfamily. ATL1/ SPG3A is expressed predominantly in the adult and fetal central nervous system. Expression of ATL1/ SPG3A in adult brain is at least 50-fold higher than in other tissues. ATL1/ SPG3A is detected predominantly in pyramidal neurons in the cerebral cortex and the hippocampus of the brain. ATL1/ SPG3A is also expressed in upper and lower motor neurons (at protein level). A distinguishing feature of ATL1/ SPG3A is its frequent early onset, raising the possibility that developmental abnormalities may be involved in its pathogenesis. Missense SPG3A mutant atlastin-1 proteins have impaired GTPase activity and may act in a dominant-negative, loss-of-function manner by forming mixed oligomers with wild-type atlastin-1. Defects in ATL1/ SPG3A 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.
Tag
GST tag
Regulatory
RUO
Host
Baculovirus-Insect Cells
Endotoxin Level
< 1.0 EU per μg of the protein as determined by the LAL method
Nature
Recombinant
Shipping
In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature.Bulk packages of recombinant proteins are provided as frozen liquid. They are shipped out with blue ice unless customers require otherwise.