Summary
Basic Information
Immunogen
TNFRSF1A (NP_001056.1, 40 a.a. ~ 149 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
Formulations & Storage [For reference only, actual COA shall prevail!]
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.
Target
Introduction
The protein encoded by this gene is a member of the TNF-receptor superfamily. This protein is one of the major receptors for the tumor necrosis factor-alpha. This receptor can activate NF-kappaB, mediate apoptosis, and function as a regulator of inflammation. Antiapoptotic protein BCL2-associated athanogene 4 (BAG4/SODD) and adaptor proteins TRADD and TRAF2 have been shown to interact with this receptor, and thus play regulatory roles in the signal transduction mediated by the receptor. Germline mutations of the extracellular domains of this receptor were found to be associated with the autosomal dominant periodic fever syndrome. The impaired receptor clearance is thought to be a mechanism of the disease. [provided by RefSeq]
Alternative Names
CD120a; FPF; MGC19588; TBP1; TNF-R; TNF-R-I; TNF-R55; TNFAR; TNFR1; TNFR55; TNFR60; p55; p55-R; p60
Function
Receptor for TNFSF2/TNF-alpha and homotrimeric TNFSF1/lymphotoxin-alpha. The adapter molecule FADD recruits caspase-8 to the activated receptor. The resulting death-inducing signaling complex (DISC) performs caspase-8 proteolytic activation which initiates the subsequent cascade of caspases (aspartate-specific cysteine proteases) mediating apoptosis. Contributes to the induction of non-cytocidal TNF effects including anti-viral state and activation of the acid sphingomyelinase.
Biological Process
Biological Process aortic valve development Source:BHF-UCL
Biological Process cellular response to mechanical stimulus Source:UniProtKB1 Publication
Biological Process cytokine-mediated signaling pathway Source:UniProtKB
Biological Process defense response to bacterium Source:Ensembl
Biological Process extrinsic apoptotic signaling pathway via death domain receptors Source:BHF-UCL1 Publication
Biological Process inflammatory response Source:UniProtKB
Biological Process intrinsic apoptotic signaling pathway in response to DNA damage Source:Ensembl
Biological Process negative regulation of cardiac muscle hypertrophy Source:Ensembl
Biological Process negative regulation of extracellular matrix constituent secretion Source:BHF-UCL
Biological Process negative regulation of inflammatory response Source:BHF-UCL1 Publication
Biological Process positive regulation of apoptotic process involved in morphogenesis Source:BHF-UCL
Biological Process positive regulation of I-kappaB kinase/NF-kappaB signaling Source:UniProtKB1 Publication
Biological Process positive regulation of inflammatory response Source:UniProtKB
Biological Process positive regulation of transcription by RNA polymerase II Source:BHF-UCL1 Publication
Biological Process positive regulation of tyrosine phosphorylation of STAT protein Source:BHF-UCL1 Publication
Biological Process prostaglandin metabolic process Source:InterPro
Biological Process protein localization to plasma membrane Source:UniProtKB1 Publication
Biological Process pulmonary valve development Source:BHF-UCL
Biological Process regulation of establishment of endothelial barrier Source:UniProtKB1 Publication
Biological Process regulation of tumor necrosis factor-mediated signaling pathway Source:Ensembl
Biological Process transcription by RNA polymerase II Source:Ensembl
Biological Process tumor necrosis factor-mediated signaling pathway Source:UniProtKB1 Publication
Cellular Location
Cell membrane
Golgi apparatus membrane
Secreted
A secreted form is produced through proteolytic processing.
Isoform 4
Secreted
Lacks a Golgi-retention motif, is not membrane bound and therefore is secreted.
Involvement in disease
Periodic fever, familial, autosomal dominant (FPF):
A hereditary periodic fever syndrome characterized by recurrent fever, abdominal pain, localized tender skin lesions and myalgia. Reactive amyloidosis is the main complication and occurs in 25% of cases.
Multiple sclerosis 5 (MS5):
A multifactorial, inflammatory, demyelinating disease of the central nervous system. Sclerotic lesions are characterized by perivascular infiltration of monocytes and lymphocytes and appear as indurated areas in pathologic specimens (sclerosis in plaques). The pathological mechanism is regarded as an autoimmune attack of the myelin sheath, mediated by both cellular and humoral immunity. Clinical manifestations include visual loss, extra-ocular movement disorders, paresthesias, loss of sensation, weakness, dysarthria, spasticity, ataxia and bladder dysfunction. Genetic and environmental factors influence susceptibility to the disease.
Topology
Extracellular: 30-211
Helical: 212-232
Cytoplasmic: 233-455
PTM
The soluble form is produced from the membrane form by proteolytic processing.
(Microbial infection) Glycosylated at Arg-376 by enteropathogenic E.coli protein NleB1 and S.typhimurium protein Ssek3: arginine GlcNAcylation prevents homotypic/heterotypic death domain interactions.