|IGHM_HUMAN » Ig mu chain C region »|
|Hydrophobic Thickness||30.8 ± 2.2 Å|
|Tilt Angle||0 ± 4°|
|Links||UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, Reactome|
|TM Segments||446-471 (446-472)|
Immune System (Reactome)
CD19, Complex: BTK:IGHM:ARI3A:CD19
|UniProt annotation for IGHM_HUMAN » Ig mu chain C region|
|FUNCTION: IgM antibodies play an important role in primary defense mechanisms. They have been shown to be involved in early recognition of external invaders like bacteria and viruses, cellular waste and modified self, as well as in recognition and elimination of precancerous and cancerous lesions. The membrane- bound form is found in the majority of normal B-cells alongside with IgD. Membrane-bound IgM induces the phosphorylation of CD79A and CD79B by the Src family of protein tyrosine kinases. It may cause death of cells by apoptosis. It is also found in soluble form, which represents about 30% of the total serum immunoglobulins where it is found almost exclusively as a homopentamer. After the antigen binds to the B-cell receptor, the secreted form is secreted in large amounts. SUBUNIT: Immunoglobulin (Ig) molecules consist of two chains, one heavy chain (which can be alpha, delta, epsilon, gamma or mu) and one light chain (which can be kappa or lambda) each consisting of a variable and a constant region. An IgM molecule contains thus a mu heavy chain combined with either a kappa or a lambda light chains. It is found almost exclusively as a homopentamer in the serum. Membrane-bound IgM molecules are non-covalently associated with heterodimer of CD79A and CD79B. DISEASE: Agammaglobulinemia 1, autosomal recessive (AGM1) OMIM: A primary immunodeficiency characterized by profoundly low or absent serum antibodies and low or absent circulating B cells due to an early block of B-cell development. Affected individuals develop severe infections in the first years of life. by mutations affecting the gene represented in this entry.|
|UniProt features for IGHM_HUMAN » Ig mu chain C region|
CHAIN <1 452 Ig mu chain C region. |
REGION 1 105 CH1.
REGION 106 217 CH2.
REGION 218 323 CH3.
REGION 324 452 CH4.
DISULFID 14 14 Interchain (with light chain).
DISULFID 28 88
DISULFID 134 197
DISULFID 214 214 Interchain (with heavy chain).
DISULFID 244 303
DISULFID 291 291 Interchain (with heavy chain of another subunit).
DISULFID 351 413
DISULFID 451 451 Interchain (with heavy chain).
|Amino Acid Sequence for IGHM_HUMAN » Ig mu chain C region|
|GSASAPTLFP LVSCENSPSD TSSVAVGCLA QDFLPDSITL SWKYKNNSDI SSTRGFPSVL RGGKYAATSQ VLLPSKDVMQ GTDEHVVCKV QHPNGNKEKN VPLPVIAELP PKVSVFVPPR DGFFGNPRKS KLICQATGFS PRQIQVSWLR EGKQVGSGVT TDQVQAEAKE SGPTTYKVTS TLTIKESDWL GQSMFTCRVD HRGLTFQQNA SSMCVPDQDT AIRVFAIPPS FASIFLTKST KLTCLVTDLT TYDSVTISWT RQNGEAVKTH TNISESHPNA TFSAVGEASI CEDDWNSGER FTCTVTHTDL PSPLKQTISR PKGVALHRPD VYLLPPAREQ LNLRESATIT CLVTGFSPAD VFVQWMQRGQ PLSPEKYVTS APMPEPQAPG RYFAHSILTV SEEEWNTGET YTCVAHEALP NRVTERTVDK STEGEVSADE EGFENLWATA STFIVLFLLS LFYSTTVTLF KVK|