Polyclonal Antibody to Paraplegin / SPG7 (Center) - Aff - Purified

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AP54017PU-N Polyclonal Antibody to Paraplegin / SPG7 (Center) - Aff - Purified

Alternate names: CAR, CMAR, PGN, Spastic paraplegia 7 protein Quantity: 0.4 ml Concentration: 0.5 mg/ml (Lot specific) Background: The SPG7 gene encodes a nuclear-encoded mitochondrial metalloprotease protein that is a member of the AAA (ATPases associated with a variety of cellular activities) protein family. Members of this protein family share an ATPase domain and have roles in diverse cellular processes including membrane trafficking, intracellular motility, organelle biogenesis, protein folding, and proteolysis. Two transcript variants encoding distinct isoforms have been identified for this gene. Mutations associated with this gene cause autosomal recessive spastic paraplegia 7. Uniprot ID: Q9UQ90 NCBI: NP_003110 GeneID: 6687 Host / Isotype: Rabbit / Ig Immunogen: Synthetic peptide - KLH conjugated corresponding to the central region (between 115-141aa) of Human Paraplegin / SPG7 Genename: SPG7 Format: State: Liquid purified Ig fraction Purification: Purified through a Protein A column followed by peptide affinity purification Buffer System: PBS with 0.09% (W/V) Sodium azide Applications: Immunohistochemistry on Paraffin Sections: 1/10-1/50. Western blot: ~1/1000. Enzyme immunoassay: 1/1000. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user. Molecular Weight: 88235 Da (calculated) Specificity: This antibody recognizes Human Paraplegin / SPG7. Other species not tested. Storage: Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. Shelf life: one year from despatch. Product Citations: Purchased from Acris: 1. Thal DR, Züchner S, Gierer S, Schulte C, Schöls L, Schüle R, et al. Abnormal Paraplegin Expression in Swollen Neurites, τ- and α-Synuclein Pathology in a Case of Hereditary Spastic Paraplegia SPG7 with an Ala510Val Mutation. Int J Mol Sci. 2015 Oct 21;16(10):25050-66. doi: 10.3390/ijms161025050. PubMed PMID: 26506339.

For research and in vitro use only. Not for diagnostic or therapeutic work. Material Safety Datasheets are available at www.acris-antibodies.com or on request. MS/20161012 1 / 2 AP54017PU-N: Polyclonal Antibody to Paraplegin / SPG7 (Center) - Aff - Purified

General Readings: 1. Warnecke T, Duning T, Schirmacher A, Mohammadi S, Schwindt W, Lohmann H, et al. A novel splice site mutation in the SPG7 gene causing widespread fiber damage in homozygous and heterozygous subjects. Mov Disord. 2010 Mar 15;25(4):413-20. doi: 10.1002/mds.22949. PubMed PMID: 20108356. 2. Augustin S, Gerdes F, Lee S, Tsai FT, Langer T, Tatsuta T. An intersubunit signaling network coordinates ATP hydrolysis by m-AAA proteases. Mol Cell. 2009 Sep 11;35(5):574-85. doi: 10.1016/j.molcel.2009.07.018. PubMed PMID: 19748354. 3. Karlberg T, van den Berg S, Hammarström M, Sagemark J, Johansson I, Holmberg- Schiavone L, et al. Crystal structure of the ATPase domain of the human AAA+ protein paraplegin/SPG7. PLoS One. 2009 Oct 20;4(10):e6975. doi: 10.1371/journal.pone.0006975. PubMed PMID: 19841671. 4. Brugman F, Scheffer H, Wokke JH, Nillesen WM, de Visser M, Aronica E, et al. Paraplegin mutations in sporadic adult-onset upper motor neuron syndromes. Neurology. 2008 Nov 4;71(19):1500-5. doi: 10.1212/01.wnl.0000319700.11606.21. Epub 2008 Sep 17. PubMed PMID: 18799786. 5. Tzoulis C, Denora PS, Santorelli FM, Bindoff LA. Hereditary spastic paraplegia caused by the novel mutation 1047insC in the SPG7 gene. J Neurol. 2008 Aug;255(8):1142-4. doi: 10.1007/s00415-008-0858-y. Epub 2008 Jun 23. PubMed PMID: 18563470. Pictures: Immunohistochemistry analysis in human brain tissue (Formalin-fixed, Paraffin-embedded) using Paraplegin / SPG7 antibody Cat.-No. AP54017PU-N, followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of SPG7 antibody for IHC; Clinical relevance has not been evaluated.

Western blot analysis in 293 cell line lysates (35ug/lane) using Paraplegin / SPG7 antibody Cat.-No. AP54017PU-N. This demonstrates the SPG7 antibody detected the SPG7 protein (arrow).

For research and in vitro use only. Not for diagnostic or therapeutic work. Material Safety Datasheets are available at www.acris-antibodies.com or on request. MS/20161012 2 / 2