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一般說明
Five endoplasmic reticulum (ER)-resident proteins have been shown to function as receptors for selective ER-phagy: FAM134B, SEC62, RTN3, CCPG1 and ATL3.1-2 These integral membrane proteins appear to partition into different sub-domains of the ER. All of them harbor a sequential peptide motif within the cytoplasmic region, enabling binding to LC3/GABARAP proteins associated with phagophore membrane.1 FAM134B (also known as RETREG1 or JK-1) is the first ER-phagy receptor to be identified.2
特異性
Anti-FAM134B antibody specifically recognizes an epitope on human FAM134B cytoplasmic domain.
應用
The antibody may be used in various immunochemical techniques including Immunoblotting (may appear as two isoforms ~55 kDa and ~40 kDa) and Immunofluorescence. Detection of the FAM134B band by Immunoblotting is specifically inhibited by the immunogen.
生化／生理作用
The protein was discovered to be involved in the pathogenesis of esophageal squamous cell carcinoma (ESCC).3-4 Loss of function mutations in FAM134B result in severe sensory and autonomic neuropathy (HSANII).5 In addition, FAM134B has been classified as an intra-membrane ER-resident protein that is characterized by the presence of a reticulon homology domain (RHD) and that is mainly located at the edges of the ER sheets.1,6 In research, FAM134B is implicated in the suppression of viral replication during Ebola, Dengue, Zika and West Nile viral infections.7-8 Non-structural viral proteases such as NS2S3 specifically cleave FAM134B, thereby subverting ER-phagy.7 Specific mutations in FAM134B and expression profiles are strongly correlated with colorectal cancers.9 Increased expression of FAM134B is also implicated with susceptibility to vascular dementia and allergic rhinitis.9-10
外觀
Supplied as a solution in 0.01 M phosphate buffered saline pH 7.4, containing 15 mM sodium azide as a preservative.
儲存和穩定性
For continuous use, store at 2-8°C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilution samples should be discarded if not used within 12 hours.
免責聲明
Unless otherwise stated in our catalog our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 1
閃點（°F）
Not applicable
閃點（°C）
Not applicable
J C Tang et al.
Clinical cancer research : an official journal of the American Association for Cancer Research, 7(6), 1539-1545 (2001-06-19)
In this study, we screened 19 esophageal squamous cell carcinomas (ESCCs) for the detection of genetic alterations using inter-simple sequence repeat PCR, a DNA fingerprinting approach. Three simple repetitive unanchored primers representing tri- and tetranucleotide repeats [(GTG)(5), (GACA)(4), and (GATA)(4)]
Nicholas J Lennemann et al.
Autophagy, 13(2), 322-332 (2017-01-20)
The endoplasmic reticulum (ER) is exploited by several diverse viruses during their infectious life cycles. Flaviviruses, including dengue virus (DENV) and Zika virus (ZIKV), utilize the ER as a source of membranes to establish their replication organelles and to facilitate
Ingo Kurth et al.
Nature genetics, 41(11), 1179-1181 (2009-10-20)
Hereditary sensory and autonomic neuropathy type II (HSAN II) leads to severe mutilations because of impaired nociception and autonomic dysfunction. Here we show that loss-of-function mutations in FAM134B, encoding a newly identified cis-Golgi protein, cause HSAN II. Fam134b knockdown results
Paolo Grumati et al.
Journal of cell science, 131(17) (2018-09-05)
Selective autophagy represents the major quality control mechanism that ensures proper turnover of exhausted or harmful organelles, among them the endoplasmic reticulum (ER), which is fragmented and delivered to the lysosome for degradation via a specific type of autophagy called
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