Application

Anti-Atlastin-1 Antibody, clone 3194 is an antibody against Atlastin-1 for use in Western Blotting, Immunocytochemistry.

Western Blotting Analysis: 10 µg/mL from a representative lot detected Atlastin-1 in 10 µg of mouse brain tissue lysate.
Immunocytochemistry Analysis: A representative lot localized Atlastin-1 immunoreactivity with that of REEP1 by dual fluorescent immunocytochemistry staining of paraformaldehyde-fixed rat cortical neurons (Park, S.H., et al. (2010). J. Clin. Invest. 120(4):1097-1110).
Immunocytochemistry Analysis: A representative lot localized overexpressed human Atlastin-1 in paraformaldehyde-fixed COS7 transfectants by fluorescent immunocytochemistry (Park, S.H., et al. (2010). J. Clin. Invest. 120(4):1097-1110).

General description

Atlastin-1 (UniProt Q8WXF7; also known as Brain-specific GTP-binding protein, GBP-3, GTP-binding protein 3, Guanine nucleotide-binding protein 3, hGBP3, Spastic paraplegia 3 protein A) is encoded by the ATL1 (also known as GBP3, HSN1D, SPG3, SPG3A) gene (Gene ID 51062) in human. Atlastin-1 is a dynamin/Mx/guanylate-binding protein superfamily member with three conserved guanylate-binding/GTPase active site motifs, P-loop (a.a. 74-81), DxxG (a.a. 146-149), and RD (217-218). Atlastin-1 is a multimeric integral membrane protein localized on ER and cis-Golgi apparatus and widely expressed in many tissues, including the smooth muscle, adrenal gland, kidney, testis, lung, and brain. Atlastin-1 interacts with the microtubule-severing ATPase spastin as well as with the DP1/Yop1p and reticulon families of ER-shaping proteins, Atlastin-1 knockdown results in decreased number of neuronal processes and impaired axon formation in cultured cortical neurons, while atlastin-1 overexpression increases total dendrite length both in vivo and in vitro. ALT1 gene mutations cause abnormal ER morphology and are linked to hereditary spastic paraplegias (HSPs), a genetically heterogeneous group of neurological disorders, includiing SPG3 (spastic paraplegia 3, autosomal dominan) and HSN1D (neuropathy, hereditary sensory, 1D), characterized by lower limb spasticity and weakness. Human atlastin-1 is a 492-amino acid protein that passes through the ER membrane twice (a.a. 450-470, 472-492), having both its N- and C-terminal ends at the cytoplasmic side (a.a. 1-449, 493-558).

Immunogen

Linear peptide corresponding to the N-terminal sequence of human Atlastin-1.

Other Notes

Concentration: Please refer to lot specific datasheet.

Physical form

Format: Purified

Quality

Evaluated by Western Blotting in human brain tissue lysate.

Western Blotting Analysis: 2.0 µg/mL of this antibody detected Atlastin-1 in 10 µg of human brain tissue lysate.

Specificity

Clone 3194 targets an N-terminal epitope present in both spliced isoforms of human Atlastin-1 reported by UniProt (Q8WXF7).

Target description

~63 kDa observed. 63.54/63.06 (human isoform 1/2), 63.52 kDa (monkey), and 63.38 kDa (mouse and rat) calculated.