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Product Details
16555-1-AP
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Published species
Human,
Mouse
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
SPG11 Fusion Protein Ag9769 (133-482 aa encoded by BC024161)
Conjugate
Form
Liquid
Concentration
0.27 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Wet ice (domestic); Dry ice (international)
Product Specific Information
Immunogen sequence: TLVGMKLLD KISSVPHGEL SCTTELLILA HHCFTLTCHM EGIIRVLQAA HMLTDNHLAP SEEYGLVVRL LTGIGRYNEM TYIFDLLHKK HYFEVLMRKK LDPSGTLKTA LLDYIKRCRP GDSEKHNMIA LCFSMCREIG ENHEAAARIQ LKLIESQPWE DSLKDGHQLK QLLLKALTLM LDAAESYAKD SCVRQAQHCQ RLTKLITLQI HFLNTGQNTM LINLGRHKLM DCILALPRFY QASIVAEAYD FVPDWAEILY QQVILKGDFN YLEEFKQQRL LKSSIFEEIS KKYKQHQPTD MVMENLKKLL TYCEDVYLYY KLAYEHKFYE IVNVLLKDPQ TGCCLKDMLA G (133-482 aa encoded by BC024161)
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Bioinformatics
Protein Aliases: Colorectal carcinoma-associated protein; Spastic paraplegia 11 protein; Spastic paraplegia 11 protein homolog; Spatacsin
Gene Aliases: 6030465E24Rik; A330015I11; ALS5; C530005A01Rik; CMT2X; KIAA1840; RGD1562529; SPG11
UniProt ID: (Human) Q96JI7, (Mouse) Q3UHA3
Entrez Gene ID: (Human) 80208, (Mouse) 214585, (Rat) 311372
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