Anti-Atlastin-1 Antibody, clone 3194

产品说明

一般描述

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).

特异性

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

免疫原

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

应用

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).

质量

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.

目标描述

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

外形

Protein G Purified
Format: Purified

其他说明

Concentration: Please refer to lot specific datasheet.

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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