Animal ID:
RC40104
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Host:
Chicken
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Reactivity:
human
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Buffers:
Purified chicken polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column and eluted out with both high and low pH buffers and neutralized immediately after elution then followed by dialysis against PBS.
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Storage:
Maintain refrigerated at 2-8ºC for up to 6 months. For long term storage store at -20ºC. Avoid repeated freeze-thaw cycles.
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Application:
Tested by peptide-specific ELISA (1:1,000).
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Immunogen:
Partial protein comprised of amino acids 241 - 402 of the human CLN5 protein.
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Accession number:
NM_006493
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Description:
The neuronal ceroid lipofuscinoses (NCL), also known as Batten disease, are a group of inherited severe neurodegenerative disorders primarily affecting children. They are characterised by the accumulation of autofluorescent storage material in many cells.
Six genes have been identified (CLN1, CLN2, CLN3, CLN5, CLN6, CLN8). Children suffer from visual failure, seizures, progressive physical and mental decline and premature death, associated with the loss of cortical neurones. CLN5 polypeptides directly interact with the CLN2 and CLN3 proteins. The molecular characterization of CLN5 revealed that it was synthesized as four precursor forms, due to usage of alternative initiator methionines in translation. All forms were targeted to lysosomes and the longest form, translated from the first potential methionine, was associated with membranes.
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Alternative Name(s):
NCL
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References:
- Vesa J, Chin MH, Oelgeschlager K, Isosomppi J, DellAngelica EC, Jalanko A, Peltonen L: Neuronal ceroid lipofuscinoses are connected at molecular level: interaction of CLN5 protein with CLN2 and CLN3. Mol. Biol. Cell 13(7): 2410-2420 (2002).
- Vesa J, Peltonen L: Mutated genes in juvenile and variant late infantile neuronal ceroid lipofuscinoses encode lysosomal proteins. Curr. Mol. Med. 2(5): 439-444 (2002). Review.
- Isosomppi J, Vesa J, Jalanko A, Peltonen L: Lysosomal localization of the neuronal ceroid lipofuscinosis CLN5 protein. Hum. Mol. Genet. 11(8): 885-891 (2002).Savukoski M, Klockars T, Holmberg V, Santavuori P, Lander ES, Peltonen L: CLN5, a novel gene encoding a putative transmembrane protein mutated in Finnish variant late infantile neuronal ceroid lipofuscinosis. Nat. Genet. 19(3): 286-288 (1998).Klockars T, Savukoski M, Isosomppi J, Laan M, Jarvela I, Petrukhin K, Palotie A, Peltonen L: Efficient construction of a physical map by fiber-FISH of the CLN5 region: refined assignment and long-range contig covering the critical region on 13q22. Genomics 35(1): 71-78 (1996). 286-288 (1998).Savukoski M, Kestila M, Williams R, Jarvela I, Sharp J, Harris J, Santavuori P, Gardiner M, Peltonen L: Defined chromosomal assignment of CLN5 demonstrates that at least four genetic loci are involved in the pathogenesis of human ceroid lipofuscinoses. Am. J. Hum. Genet. 55(4): 695-701 (1994).
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For Research Use Only. Not for Diagnostic or Therapeutic Use.
Purchase does not include or carry any right to resell or transfer this product either as a stand-alone product or as a component of another product. Any use of this product other than the permitted use without the express written authorization of Orbigen, Inc. is strictly prohibited
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