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FLASH GENE

Symbol

C4BPA

contributors: mct - updated : 24-12-2013

HGNC name	complement component 4 binding protein, alpha
HGNC id	1325

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

DNA

TYPE	functioning gene
STRUCTURE	40.73 kb     12 Exon(s)

Genomic sequence alignment details
10 Kb 5' upstream gene genomic sequence study

MAPPING

cloned

Y

linked

Y

status

confirmed

Map

see RCA@

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_000715 12 - 2256 NP_000706 - 597 - - a 48 aa signal peptide of 5.4 kda a 549 aa mature peptide of 61.7 kda

EXPRESSION

Type

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol Digestive liver       predominantly Reproductive female system uterus cervix   moderately Respiratory lung       moderately Urinary bladder       highly

cell lineage
cell lines
fluid/secretion	plasma

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	eight short consensus repeats, sushi structure (see F13B, SRCP1) region common to Ba region in factor B
	four complement control protein domains

conjugated

GlycoP

HOMOLOGY

interspecies

homolog to rattus C4bpa (59.6 pc)

Homologene

FAMILY

CATEGORY

immunity/defense , regulatory

SUBCELLULAR LOCALIZATION	extracellular
	plasma membrane
	intracellular
	intracellular,cytoplasm
	intracellular,nucleus

basic FUNCTION	playing a role of regulator of C3 convertase of classical pathway
	facilitating the factor I-mediated proteolytic cleavage of the active forms of complement effectors CR1/C4B into their inactive forms
	controlling the classical pathway of complement activation
	hydrolyzing the complement fragment C4b
	accelerating the degradation of the C4bC2a complex (C3 convertase) by dissociating the complement fragment C2a
	soluble, multiple-subunit inhibitor of complement that circulates in blood
	is recruited to the S. aureus surface where it functions to inhibit C4 complement effectors

CELLULAR PROCESS

PHYSIOLOGICAL PROCESS

immunity/defense

text	innate immune response
	complement activation, classical pathway

PATHWAY

metabolism

signaling

a component

complexing with C4BPB

INTERACTION

DNA	multivalent interaction with DNA or surface bound glycosaminoglycans

RNA

small molecule

protein	C4, alpha chain
	binding as a cofactor to C3b/C4b inactivator (C3bINA)
	interacting with anticoagulant protein S and with serum amyloid P component
	PTX3 binds to the classical and lectin pathway regulator C4b-binding protein (C4BPA)
	PRELP interact with the complement inhibitor C4B-binding protein, C4BPB, and C4BPA
	major inhibitor of the classical complement and lectin pathway

cell & other

REGULATION

ASSOCIATED DISORDERS

corresponding disease(s)

Susceptibility

to idiopathic, recurrent, spontaneous pregnancy loss

Variant & Polymorphism other	R120H, I126T, and the G423T mutations affected the expression level and/or the ability of recombinant C4BP to serve as cofactor for factor I, and are associated with idiopathic, recurrent, spontaneous pregnancy loss

Candidate gene

Marker

Therapy target

ANIMAL & CELL MODELS