Human ATL1 / SPG3A / Atlastin-1 Protein (GST tag) – PKSH031549

Recombinant Human ATL1 / SPG3A / Atlastin-1 Protein (GST tag)

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.

Synonym(s) : ATL1 , AD-FSP , FSP1 , GBP3 , SPG3 , SPG3A , atlastin1

Species(s) : Human

Protein Expression Host : Baculovirus-Insect Cells

Protein Construction : A DNA sequence encoding the human SPG3A (NP_056999.2) (Met 1-Thr 447) was fused with the GST tag at the N-terminus.

Predicted N-terminal amino acid : Met

Molecular weight/mass : The recombinant human SPG3A/GST chimera consists of 671 amino acids and and has a calculated molecular mass of 77 kDa. It migrates as an approximately 66 kDa band in SDS-PAGE under reducing conditions.

Protein Construction : A DNA sequence encoding the human SPG3A (NP_056999.2) (Met 1-Thr 447) was fused with the GST tag at the N-terminus.

Formulation : Lyophilized from sterile 50mM Tris, 100mM NaCl, 0.5mM PMSF, 0.5mM EDTA, 0.5mM GSH, pH 8.0

Purity : > 80 % as determined by SDS-PAGE

Endotoxin level : < 1.0 EU per μg of the protein as determined by the LAL method

Stability : Samples are stable for up to twelve months from date of receipt at -70°C

Shipping conditions : 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.

Reconstitution : Please refer to the printed manual for detailed information.

Manual & Datasheet