Recombinant human AFG3-like protein 2 protein

Relevance: ATP-dependent protease which is essential for axonal development Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28) [MIM:610246]. It is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment.
Product Info: GST-tagged
Source: E.coli derived
Purity: 95%
Image:
Tested applications: SDS-PAGE, ELISA, Western blot
Storage Buffer: 6M guanidine hydrochloride,20mM Tris
Storage: Store at -20?, for extended storage, conserve at -20? or -80?.
Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4? for up to one week.
AA sequence: ADPLLKVSIIPRGKGLGYAQYLPKEQYLYTKEQLLDRMCMTLGGRVSEEIFFGRITTGAQDDLRKVTQSAYAQIVQFGMNEKVGQISFDLPRQGDMVLEKPYSEATARLIDDEVRILINDAYKRTVALLTEKKADVEKVALLLLEKEVLDKNDMVELLGPRPFAEKSTYEEFVEGTGSLDEDTSLPEGLKDWNKEREKEKEEPPGEKVAN
Referrences: [1] "Identification and characterization of AFG3L2, a novel paraplegin-related gene." Banfi S., Bassi M.T., Andolfi G., Marchitiello A., Zanotta S., Ballabio A., Casari G., Franco B. Genomics 59:51-58(1999) [PubMed: 10395799] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], SUBCELLULAR LOCATION. [2] "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Eye. [3] "Lysine acetylation targets protein complexes and co-regulates major cellular functions." Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T., Olsen J.V., Mann M. Science 325:834-840(2009) [PubMed: 19608861] [Abstract] Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-306 AND LYS-543, MASS SPECTROMETRY. [4] "Initial characterization of the human central proteome." Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J. BMC Syst. Biol. 5:17-17(2011) [PubMed: 21269460] [Abstract] Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
Alias: Paraplegin-like protein.

Информация для заказа

Область использования: Рекомбинантные белки Cusabio	Производство:	Cusabio
	Метод:
	Объем:	500 мкг
	Кат. номер:	CSB-RP040054h
Цена (с НДС 20%):	по запросу	В корзину
Наименование: Recombinant human AFG3-like protein 2 protein. Примечание: дополнительная информация (на английском языке).