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CD126; interleukin-6 receptor subunit alpha; IL-6R-alpha; IL-6R 1; membrane glycoprotein 80; gp80 (IL6R)

Function: - IL-6 receptor subunit - binds to IL6 with low affinity, but does not transduce a signal - signal activation requires association with gp130 - external domain of IL-6 receptor subunit interacts with a cell surface protein, gp130, that functions as a signal transducer - soluble form may also be released from the membrane by proteolysis - low concentration of a soluble form of IL6R acts as an agonist of IL6 activity - soluble IL6R activates osteoclasts & thus provokes bone reabsorbtion Structure: - the two fibronectin type-3-like domains, contained in the N-terminal part, form together a cytokine-binding domain - the WSXWS motif appears to be necessary for proper protein folding, efficient intracellular transport & cell-surface receptor binding - belongs to the type 1 cytokine receptor family, type 3 subfamily - contains 1 fibronectin F3 module - contains 1 Ig-like C2-type domains (immunoglobulin-like) Compartment: - isoform 1: basolateral cell membrane - isoform 2: secreted soluble IL6R may also be present in serum Alternative splicing: named isoforms=2 Expression: - isoform 2 is expressed in peripheral blood mononuclear cells & weakly found in urine & serum Pathology: - soluble IL6R may play a role in patients with multiple myeloma

Related

CD126 blasts in blood CD126 blasts in bone marrow interleukin-6; IL-6; B-cell stimulatory factor 2; BSF-2; CTL differentiation factor; CDF; hybridoma growth factor; interferon beta-2; IFN-beta-2 (IL6, IFNB2)

General

cluster-of-differentiation antigen; cluster designation antigen; CD antigen hematopoietin receptor superfamily protein subunit

Properties

SIZE: entity length = 468 aa MW = 52 kD COMPARTMENT: cellular membrane MOTIF: signal sequence {1-19} cysteine residue {C25} MODIFICATION: cysteine residue {C193} immunoglobulin superfamily domain {26-112} MOTIF: cysteine residue {C47} MODIFICATION: cysteine residue {C96} N-glycosylation site {N55} N-glycosylation site {N93} cysteine residue {C96} MODIFICATION: cysteine residue {C47} cysteine residue {C121} MODIFICATION: cysteine residue {C132} cysteine residue {C132} MODIFICATION: cysteine residue {C121} cysteine residue {C165} MODIFICATION: cysteine residue {C176} cysteine residue {C176} MODIFICATION: cysteine residue {C165} cysteine residue {C193} MODIFICATION: cysteine residue {C25} fibronectin type III domain or F3 module {216-311} MOTIF: N-glycosylation site {N221} asparagine residue {245} WSXWS motif {303-307} transmembrane domain {366-386}

Database Correlations

OMIM 147880 MORBIDMAP 147880 UniProt P08887 PFAM correlations Entrez Gene 3570 Kegg hsa:3570

References

  1. Ihle JN et al Signaling by the cytokine receptor superfamily: JAKs and STATs TIBS 19:222 1994 PMID: 8048164
  2. Itoh N, Yonehara S, Schreurs J, Gorman DM, Maruyama K, Ishii A, Yahara I, Arai K, Miyajima A. Cloning of an interleukin-3 receptor gene: a member of a distinct receptor gene family. Science. 1990 Jan 19;247(4940):324-7. PMID: 2404337
  3. Bataille R, Harousseau JL. Multiple myeloma. N Engl J Med. 1997 Jun 5;336(23):1657-64. Review. PMID: 9171069
  4. UniProt :accession P08887

Component-of

interleukin-6 receptor