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Mouse Anti-SPG21 Recombinant Antibody (CBXS-4375) (CBMAB-S1618-CQ)

This product is a mouse antibody that recognizes SPG21. The antibody CBXS-4375 can be used for immunoassay techniques such as: ELISA.
See all SPG21 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXS-4375
Antibody Isotype
IgG2a, κ
Application
ELISA

Basic Information

Immunogen
SPG21 (NP_057714, 211 a.a.-306 a.a) partial recombinant protein with GST tag
Specificity
Human
Antibody Isotype
IgG2a, 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
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
spastic paraplegia 21 (autosomal recessive, Mast syndrome)
Introduction
The protein encoded by this gene binds to the hydrophobic C-terminal amino acids of CD4 which are involved in repression of T cell activation. The interaction with CD4 is mediated by the noncatalytic alpha/beta hydrolase fold domain of this protein. It is thus proposed that this gene product modulates the stimulatory activity of CD4. Mutations in this gene are associated with autosomal recessive spastic paraplegia 21 (SPG21), also known as mast syndrome. Alternative splicing results in multiple transcript variants.
Entrez Gene ID
51324
UniProt ID
Q9NZD8
Alternative Names
SPG21, Maspardin; Spastic Paraplegia 21 (Autosomal Recessive, Mast Syndrome); Acid Cluster Protein 33; ACP33; Spastic Paraplegia 21 Autosomal Recessive Mast Syndrome Protein; Spastic Paraplegia 21 Protein;
Function
May play a role as a negative regulatory factor in CD4-dependent T-cell activation.
Biological Process
Antigen receptor-mediated signaling pathway
Cellular Location
Cytoplasm, cytosol
Membrane
Endosome membrane
Golgi apparatus, trans-Golgi network membrane
Partially localized in the cytosol but also accumulated on an intracellular vesicular compartment. Colocalizes with CD4 on endosomal/trans-Golgi network.
Involvement in disease
Spastic paraplegia 21, autosomal recessive (SPG21):
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. SPG21 is associated with dementia and other central nervous system abnormalities. Subtle childhood abnormalities may be present, but the main features develop in early adulthood. The disease is slowly progressive, and cerebellar and extrapyramidal signs are also found in patients with advanced disease. Patients have a thin corpus callosum and white-matter abnormalities.
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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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