Application

The antibody may be used in various immunochemical techniques including Immunoblotting (~55 kDa) and Immunofluorescence. Detection of the FAM134B band by Immunoblotting is specifically inhibited by the immunogen.

Biochem/physiol Actions

FAM134B 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

Disclaimer

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.

General description

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

Physical form

Supplied as a solution in 0.01 M phosphate buffered saline pH 7.4, containing 15 mM sodium azide as a preservative.

Specificity

Anti-FAM134B (lumenal) antibody specifically recognizes an epitope on human FAM134B ER luminal domain.

Storage and Stability

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.