Full Product Name
SPG11 Antibody
Product Synonym Names
Spastic paraplegia 11, colorectal carcinoma-associated protein, spatacsin
Product Gene Name
anti-SPG11 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Affinity chromatography purified via peptide column
Form/Format
Supplied in PBS containing 0.02% sodium azide.
Immunogen Description
Raised against a 15 amino acid peptide of human SPG11.
Preparation and Storage
Can be stored at -20 degree C, stable for one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Other Notes
Small volumes of anti-SPG11 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-SPG11 antibody
Hereditary spastic paraplegias (HSPs) are genetically and phenotypically heterogeneous disorders. Spastic paraplegia with thinning of the corpus callosum (ARHSP-TCC) is a relatively frequent form of complicated hereditary spastic paraplegia (cHSP) in which mental retardation and muscle stiffness at onset are followed by slowly progressive paraparesis and cognitive deterioration. Mutations of the SPG11 gene encoding the spatacsin protein have been identified as a major cause of HSP-TCC. Spatacsin is a potential transmembrane protein that is phosphorylated upon DNA damage. It is expressed in all structures of the brain, with a high expression in the cerebellum. SPG11 mutations may occur more frequently in familial than sporadic forms of cHSP without TCC. Kjellin syndrome is found to be associated with mutations in not only the SPG15 gene but also SPG11 gene. Recent studies show Parkinsonism may initiate SPG11-linked HSP TCC and that SPG11 may cause juvenile Parkinsonism.
Product Categories/Family for anti-SPG11 antibody
Total protein Ab
Applications Tested/Suitable for anti-SPG11 antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
Western Blot (WB) of anti-SPG11 antibody
Western blot analysis of SPG11 in mouse heart tissue lysate with SPG11 antibody at (A) 0.5 and (B) 1 ug/mL.
Immunohistochemistry (IHC) of anti-SPG11 antibody
Immunohistochemistry of SPG11 in mouse brain tissue with SPG11 antibody at 2.5 ug/mL.
NCBI/Uniprot data below describe general gene information for SPG11. It may not necessarily be applicable to this product.
NCBI Accession #
AAI53880
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UniProt Secondary Accession #
Q4VC11; Q58G86; Q69YG6; Q6NW01; Q8N270; Q8TBU9; Q9H734; A8KAX9; B9EK60; F5H3N6[Other Products]
UniProt Related Accession #
Q96JI7[Other Products]
Molecular Weight
266,643 Da
NCBI Official Full Name
Spastic paraplegia 11 (autosomal recessive)
NCBI Official Synonym Full Names
spastic paraplegia 11 (autosomal recessive)
NCBI Official Symbol
SPG11??[Similar Products]
NCBI Official Synonym Symbols
KIAA1840
??[Similar Products]
NCBI Protein Information
spatacsin
UniProt Protein Name
Spatacsin
UniProt Synonym Protein Names
Colorectal carcinoma-associated protein; Spastic paraplegia 11 protein
UniProt Gene Name
SPG11??[Similar Products]
UniProt Synonym Gene Names
KIAA1840??[Similar Products]
UniProt Entry Name
SPTCS_HUMAN
NCBI Summary for SPG11
The protein encoded by this gene is a potential transmembrane protein that is phosphorylated upon DNA damage. Defects in this gene are a cause of spastic paraplegia type 11 (SPG11). Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2009]
UniProt Comments for SPG11
SPG11: Defects in SPG11 are the cause of spastic paraplegia autosomal recessive type 11 (SPG11). Spastic paraplegia is 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. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Membrane protein, multi-pass; Membrane protein, integral
Chromosomal Location of Human Ortholog: 15q14
Cellular Component: lysosomal membrane; cytoplasm; nucleolus; integral to membrane; plasma membrane; cytoplasmic vesicle; cytosol
Molecular Function: protein binding
Disease: Spastic Paraplegia 11, Autosomal Recessive
Research Articles on SPG11
1. spastizin and spatacsin were essential components for the initiation of lysosomal tubulation. Together, these results link dysfunction of the autophagy/lysosomal biogenesis machinery to neurodegeneration.
Precautions
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Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
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