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The size of either C4A or C4B gene can be 21 kb (long, L) or 14.6 kb (short, S). Also, the long C4 gene uniquely contains a retrovirus HERV-K(C4) in its intron 9 that imposes transcription of an extra 6.36 kb, hence the “longer” string of gene. [2] [14] Thus, C4 genes have a complex pattern of variation in gene size, copy number, and ...
For example, if and individual has normal C3/C4 values but a decreased CH50, that can indicate a terminal complement pathway deficiency while if one has low C3 and CH50 values that can indicate an autoimmune condition such as systemic lupus erythematosus.
Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. [4] Because of redundancies in the immune system , many complement disorders are never diagnosed.
Complement tests C4 FB C3 CH50 ... ↓ ↓ SLE: ↑ ↑ ↑ ↑ inflammation: Complement 4 deficiency is a genetic condition affecting complement component 4. [1] It ...
12268 Ensembl ENSG00000244207 ENSG00000206340 ENSG00000244731 ENSG00000227746 ENSMUSG00000073418 UniProt P0C0L4 P01029 RefSeq (mRNA) NM_001252204 NM_007293 NM_009780 RefSeq (protein) NP_001239133 NP_009224 NP_033910 Location (UCSC) Chr 6: 31.98 – 32 Mb Chr 17: 34.95 – 34.96 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Complement C4-A is a kind of the Complement component 4 ...
Anaphylatoxins, or complement peptides, are fragments (C3a, C4a and C5a) that are produced as part of the activation of the complement system. [2] Complement components C3, C4 and C5 are large glycoproteins that have important functions in the immune response and host defense. [ 3 ]
Beta-2 comprises C3 (complement protein 3). It is raised in the acute phase response. Depression of C3 occurs in autoimmune disorders as the complement system is activated and the C3 becomes bound to immune complexes and removed from serum. Fibrinogen, a beta-2 protein, is found in normal plasma but absent in normal serum.
Complement control proteins are proteins that interact with components of the complement system. The complement system is tightly regulated by a network of proteins known as "regulators of complement activation (RCA)" that help distinguish target cells as "self" or "non-self."