Mouse Anti-SPG3A Recombinant Antibody (1B9) (CBMAB-A8533-LY)

Name: Mouse Anti-SPG3A Recombinant Antibody (1B9)
SKU: CBMAB-A8533-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

1B9

Application

WB, ELISA

Immunogen

SPG3A (NP_056999, 1 a.a. ~ 100 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Specificity

Human, Rat

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

Purity

> 95% Purity determined by SDS-PAGE.

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

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. [provided by RefSeq]

Entrez Gene ID

Human	51062
Rat	362750

UniProt ID

Human	Q8WXF7
Rat	Q6PST4

Alternative Names

AD-FSP; FSP1; GBP3; SPG3; SPG3A; atlastin1

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Related Products

Mouse Anti-SPG3A Recombinant Antibody (1B11) (CAT#: CBMAB-A8532-LY)

Isotype control

For research use only. Not intended for any clinical use.

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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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