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Mouse Anti-ATL1 Recombinant Antibody (CBXS-2684) (CBMAB-S5436-CQ)

This product is a mouse antibody that recognizes ATL1. The antibody CBXS-2684 can be used for immunoassay techniques such as: ELISA, WB.
See all ATL1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXS-2684
Antibody Isotype
IgG1, κ
Application
ELISA, WB

Basic Information

Immunogen
SPG3A (NP_056999, 1aa-100aa) partial recombinant protein with GST tag.
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.4
Preservative
None
Concentration
Batch dependent
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Atlastin GTPase 1
Introduction
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.
Entrez Gene ID
51062
UniProt ID
Q8WXF7
Alternative Names
Atlastin GTPase 1; Guanine Nucleotide-Binding Protein 3; Brain-Specific GTP-Binding Protein; Spastic Paraplegia 3 Protein A; GTP-Binding Protein 3; SPG3A; GBP-3; HGBP3; GBP3; Spastic Paraplegia 3A (Autosomal Dominant);
Function
GTPase tethering membranes through formation of trans-homooligomers and mediating homotypic fusion of endoplasmic reticulum membranes. Functions in endoplasmic reticulum tubular network biogenesis (PubMed:27619977).
May also regulate Golgi biogenesis. May regulate axonal development.
Biological Process
Axonogenesis Source: UniProtKB
Endoplasmic reticulum organization Source: UniProtKB
Endoplasmic reticulum tubular network membrane organization Source: UniProtKB
Protein homooligomerization Source: UniProtKB
Cellular Location
Golgi apparatus membrane; Endoplasmic reticulum membrane; Axon. Localizes to endoplasmic reticulum tubular network (PubMed:27619977).
Involvement in disease
Spastic paraplegia 3, autosomal dominant (SPG3): A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body.
Neuropathy, hereditary sensory, 1D (HSN1D): 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.
Topology
Cytoplasmic: 1-449 aa
Helical: 450-470 aa
Lumenal: 471 aa
Helical: 472-492 aa
Cytoplasmic: 493-558 aa
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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