Mouse SPG3A Monoclonal Antibody | anti-SPG3A antibody

SPG3A (atlastin GTPase 1, AD-FSP, FSP1, GBP3, SPG3, SPG3A, atlastin1) (APC)

Cat. Num. AAA6168236

• Applications: ELISA, Western Blot
• Purity: Purified
• Synonyms: SPG3A; Monoclonal Antibody; SPG3A (atlastin GTPase 1; AD-FSP; FSP1; GBP3; SPG3; atlastin1) (APC); atlastin GTPase 1; atlastin1; anti-SPG3A antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG1,k
• Clone Number: 1B11
• Specificity: Recognizes SPG3A.
• Purity/Purification: Purified
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).
• Sequence Length: 558

• Applicable Applications for anti-SPG3A antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: SPG3A (NP_056999, 1aa-100aa) partial recombinant protein with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: MAKNRRDRNSWGGFSEKTYEWSSEEEEPVKKAGPVQVLIVKDDHSFELDETALNRILLSEAVRDKEVVAVSVAGAFRKGKSFLMDFMLRYMYNQESVDWV
• Conjugate: APC
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(SPG3A monoclonal antibody (M10), clone 1B11. Western Blot analysis of SPG3A expression in IMR-32.)

Western Blot (WB)

(SPG3A monoclonal antibody (M10), clone 1B11. Western Blot analysis of SPG3A expression in PC-12.)

Related Product Information for anti-SPG3A antibody

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]

Product Categories/Family for anti-SPG3A antibody

Antibodies; GTPase; Rab; Ras; Rho Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 19923445
• NCBI Accession #: NP_056999
• NCBI GenBank Nucleotide #: NP_056999.2
• UniProt Accession #: Q8WXF7
• NCBI Official Full Name: atlastin-1 isoform a
• UniProt Protein Name: Atlastin-1
• UniProt Gene Name: ATL1
• UniProt Synonym Gene Names: GBP3; SPG3A; GBP-3; hGBP3

Uniprot Description

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

Chromosomal Location of Human Ortholog: 14q22.1

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

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

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

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