Spastizin (Zinc Finger FYVE Domain-containing Protein 26, FYVE Domain-containing Centrosomal Protein, FYVE-CENT, ZFYVE26, KIAA0321)

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 in which mental retardation and muscle stiffness at onset are followed by slowly progressive paraparesis and cognitive deterioration. SPG15 is the second gene known to be responsible for ARHSP-TCC in the Italian population. Mutations in this gene are associated with autosomal recessive spastic paraplegia-15. SPG15 encodes a protein containing a FYVE zinc finger binding domain which is thought to target these proteins to membrane lipids through interaction with phospholipids in the membrane. SPG15 mRNA is widely distributed in human tissues, as well as in rat embryos, suggesting a possible role for this protein during embryonic development. SPG15 co-localizes partially with endoplasmic reticulum and endosome markers, suggesting a role in intracellular trafficking. Multiple isoforms of SPG15 are known to exist.\n\nApplications:\nSuitable for use in Immunofluorescence, ELISA and Western Blot. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 0.5-1ug/ml\nImmunofluorescence: 20ug/ml\nOptimal dilutions to be determined by the researcher.\n\nPositive Control:\nRat Heart Tissue Lysate \n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

146388

Size

100ug

Applications

ELISA, IF, WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat

Form

Supplied as a liquid in PBS, 0.02% sodium azide.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

References

1. Hughes CA, Byrne PC, Webb S, et al. SPG15, a new locus for autosomal recessive complicated HSP on chromosome 14q. Neurology 2001; 56:1230-3. 2. Denora PS, Muglia M, Casali C, et al. Spastic paraplegia with thinning of the corpus callosum and white matter abnormalities: further mutations and relative frequency in ZFYVE26/SPG15 in the Italian population. J. Neurol. Sci. 2009; 277:22-5. 3. Hanein S, Martin E, Boukhris A, et al. Identification of the SPG15 gene, encoding spastizin, as a frequent cause of complicated autosomal-recessive spastic paraplegia, including Kjellin syndrome. Am. J. Hum. Genet. 2008; 82:992-1002. 4. Boukhris A, Feki I, Denis E, et al. Spastic paraplegia 15: linkage and clinical description of three Tunisian families. Mov. Disord. 2008; 23:429-33.

Additional Info

Recognizes human SPG15. Species Crossreactivity: mouse and rat.