C4b complement C4B (Chido blood group) [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: C4bprovided by MGI
Official Full Name: complement C4B (Chido blood group)provided by MGI
Primary source: MGI:MGI:88228
See related: Ensembl:ENSMUSG00000073418 AllianceGenome:MGI:88228
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: C4; Ss
Summary: Predicted to enable carbohydrate binding activity and complement component C1q complex binding activity. Acts upstream of or within complement activation and immunoglobulin mediated immune response. Located in extracellular space. Is expressed in several structures, including hemolymphoid system; liver; reproductive system; retina; and yolk sac. Used to study systemic lupus erythematosus. Human ortholog(s) of this gene implicated in autistic disorder; autoimmune disease (multiple); complement deficiency (multiple); and renal cell carcinoma. Orthologous to several human genes including C4A (complement C4A (Chido/Rodgers blood group)) and C4B (complement C4B (Chido/Rodgers blood group)). [provided by Alliance of Genome Resources, Nov 2024]
Expression: Biased expression in liver adult (RPKM 237.2), subcutaneous fat pad adult (RPKM 85.1) and 9 other tissues See more
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Genomic context

Location:: 17 B1; 17 18.29 cM

Exon count:: 42

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	17	NC_000083.7 (34947249..34962876, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	17	NC_000083.6 (34728139..34743902, complement)

Chromosome 17 - NC_000083.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

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Expression

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See details

Project title: Mouse ENCODE transcriptome data Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

These data show that Borrelia burgdorferi OspC inhibits the classical and lectin complement pathways and competes with complement protein C2 for C4b binding.
Title: Borrelia burgdorferi outer surface protein C (OspC) binds complement component C4b and confers bloodstream survival.
C4 mediated synapse elimination during postnatal development
Title: Schizophrenia risk from complex variation of complement component 4.
role in suppressing autoantibody production elicited by S. pneumoniae antigens
Title: C4 Deficiency is a predisposing factor for Streptococcus pneumoniae-induced autoantibody production.
Data indicate complement C3 deposition on peanut extract (PE) was abolished in immunoglobulin- and C4-deficient sera.
Title: Role of complement in a murine model of peanut-induced anaphylaxis.
a deficiency in C4b does not alter radiation-induced lung disease in the C57BL/6 mouse model
Title: Genetic deficiency in complement component 4b does not alter radiation-induced lung disease in mice.
C3 and C4 bind to collagen and elastin in the vascular wall and have a potential role in vascular stiffness and atherosclerosis
Title: Complement proteins C3 and C4 bind to collagen and elastin in the vascular wall: a potential role in vascular stiffness and atherosclerosis.
Increased transcription of the complement C4 gene in the Rag1(-/-) intestine.
Title: Enhanced transcription of complement and coagulation genes in the absence of adaptive immunity.
The increased mortality of C4(-/-) or Factor B(-/-) mice indicates that these two pathways of complement activation are required for survival in mousepox.
Title: Surviving mousepox infection requires the complement system.
In comparison with C57BL/6 mice, MRL/1pr mice had depressed C4 levels as early as 3 weeks of age.
Title: Analysis of C4 and the C4 binding protein in the MRL/lpr mouse.
Complement C4 provides an important protective role against the development of systemic lupus erythematosus.
Title: Complement C4 is protective for lupus disease independent of C3.

Submit: New GeneRIF Correction See all GeneRIFs (16)

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (7) 1 citation
Targeted (1) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

C4 (e-PCR), detects polymorphism
- UniSTS:257038

D17Mit31 (e-PCR), detects polymorphism
- UniSTS:128080

D17Mit32 (e-PCR), detects polymorphism
- UniSTS:128081

D17Mit33 (e-PCR), detects polymorphism
- UniSTS:128082

D17Mit34 (e-PCR), detects polymorphism
- UniSTS:128083

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables carbohydrate binding	ISO Inferred from Sequence Orthology more info
enables complement binding	ISO Inferred from Sequence Orthology more info
enables complement component C1q complex binding	ISO Inferred from Sequence Orthology more info
enables endopeptidase inhibitor activity	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
involved_in complement activation	IBA Inferred from Biological aspect of Ancestor more info
acts_upstream_of_or_within complement activation	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in complement activation, classical pathway	IEA Inferred from Electronic Annotation more info
acts_upstream_of_or_within immunoglobulin mediated immune response	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in inflammatory response	IEA Inferred from Electronic Annotation more info
involved_in innate immune response	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
located_in axon	IEA Inferred from Electronic Annotation more info
located_in axon	ISO Inferred from Sequence Orthology more info
located_in dendrite	IEA Inferred from Electronic Annotation more info
located_in dendrite	ISO Inferred from Sequence Orthology more info
located_in extracellular region	ISO Inferred from Sequence Orthology more info
located_in extracellular space	HDA more info	PubMed
is_active_in extracellular space	IBA Inferred from Biological aspect of Ancestor more info
located_in extracellular space	IDA Inferred from Direct Assay more info	PubMed
located_in extracellular space	ISO Inferred from Sequence Orthology more info
located_in neuronal cell body	ISO Inferred from Sequence Orthology more info
located_in symbiont cell surface	ISO Inferred from Sequence Orthology more info
located_in synapse	IEA Inferred from Electronic Annotation more info
located_in synapse	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: complement C4-B

Names: complement component 4 (within H-2S); complement component 4B (Chido blood group); complement component 4B (Childo blood group)

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_009780.2 → NP_033910.2 complement C4-B precursor

See identical proteins and their annotated locations for NP_033910.2

Status: VALIDATED

Source sequence(s)

AK128974, AK157954, AK158256, AK158475, BC067394, BY785731, CA459454, K02798

Consensus CDS

CCDS28657.1

UniProtKB/Swiss-Prot

E9QKK7, O70346, P01029, Q31201, Q3TYY1, Q3TZC9, Q61372, Q61859, Q62353, Q6NWV8

UniProtKB/TrEMBL

B2RWX2

Related

ENSMUSP00000069418.9, ENSMUST00000069507.9

Conserved Domains (8) summary

cd00017
Location:686 → 752

ANATO; Anaphylatoxin homologous domain; C3a, C4a and C5a anaphylatoxins are protein fragments generated enzymatically in serum during activation of complement molecules C3, C4, and C5. They induce smooth muscle contraction. These fragments are homologous to ...

cd02896
Location:992 → 1311

complement_C3_C4_C5; Proteins similar to C3, C4 and C5 of vertebrate complement. The vertebrate complement system, comprised of a large number of distinct plasma proteins, is an effector of both the acquired and innate immune systems. The point of convergence of the ...

pfam00207
Location:780 → 867

A2M; Alpha-2-macroglobulin family

pfam01835
Location:139 → 231

A2M_N; MG2 domain

pfam07677
Location:1475 → 1564

A2M_recep; A-macroglobulin receptor

pfam07678
Location:1047 → 1313

A2M_comp; A-macroglobulin complement component

pfam07703
Location:475 → 608

A2M_N_2; Alpha-2-macroglobulin family N-terminal region

cl02512
Location:1582 → 1736

NTR_like; NTR_like domain; a beta barrel with an oligosaccharide/oligonucleotide-binding fold found in netrins, complement proteins, tissue inhibitors of metalloproteases (TIMP), and procollagen C-proteinase enhancers (PCOLCE), amongst others. In netrins, the ...

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

NC_000083.7 Reference GRCm39 C57BL/6J

Range

34947249..34962876 complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_006523532.5 → XP_006523595.1 complement C4-B isoform X2

UniProtKB/TrEMBL

B2RWX2

Conserved Domains (8) summary

cd00017
Location:686 → 752

ANATO; Anaphylatoxin homologous domain; C3a, C4a and C5a anaphylatoxins are protein fragments generated enzymatically in serum during activation of complement molecules C3, C4, and C5. They induce smooth muscle contraction. These fragments are homologous to ...

pfam00207
Location:780 → 867

A2M; Alpha-2-macroglobulin family

pfam07677
Location:1455 → 1544

A2M_recep; A-macroglobulin receptor

pfam07678
Location:978 → 1311

A2M_comp; A-macroglobulin complement component

pfam17791
Location:233 → 318

MG3; Macroglobulin domain MG3

pfam01835
Location:139 → 231

A2M_N; MG2 domain

pfam07703
Location:470 → 609

A2M_N_2; Alpha-2-macroglobulin family N-terminal region

cl02512
Location:1562 → 1716

NTR_like; NTR_like domain; a beta barrel with an oligosaccharide/oligonucleotide-binding fold found in netrins, complement proteins, tissue inhibitors of metalloproteases (TIMP), and procollagen C-proteinase enhancers (PCOLCE), amongst others. In netrins, the ...
XM_006523531.5 → XP_006523594.1 complement C4-B isoform X1

UniProtKB/TrEMBL

B2RWX2

Conserved Domains (8) summary

cd00017
Location:686 → 752

ANATO; Anaphylatoxin homologous domain; C3a, C4a and C5a anaphylatoxins are protein fragments generated enzymatically in serum during activation of complement molecules C3, C4, and C5. They induce smooth muscle contraction. These fragments are homologous to ...

pfam00207
Location:780 → 867

A2M; Alpha-2-macroglobulin family

pfam07677
Location:1475 → 1564

A2M_recep; A-macroglobulin receptor

pfam07678
Location:978 → 1311

A2M_comp; A-macroglobulin complement component

pfam17791
Location:233 → 318

MG3; Macroglobulin domain MG3

pfam01835
Location:139 → 231

A2M_N; MG2 domain

pfam07703
Location:470 → 609

A2M_N_2; Alpha-2-macroglobulin family N-terminal region

cl02512
Location:1582 → 1736

NTR_like; NTR_like domain; a beta barrel with an oligosaccharide/oligonucleotide-binding fold found in netrins, complement proteins, tissue inhibitors of metalloproteases (TIMP), and procollagen C-proteinase enhancers (PCOLCE), amongst others. In netrins, the ...
XM_030249459.1 → XP_030105319.1 complement C4-B isoform X3

UniProtKB/TrEMBL

B2RWX2

Conserved Domains (8) summary

cd00017
Location:686 → 752

ANATO; Anaphylatoxin homologous domain; C3a, C4a and C5a anaphylatoxins are protein fragments generated enzymatically in serum during activation of complement molecules C3, C4, and C5. They induce smooth muscle contraction. These fragments are homologous to ...

pfam00207
Location:780 → 867

A2M; Alpha-2-macroglobulin family

pfam07677
Location:1455 → 1544

A2M_recep; A-macroglobulin receptor

pfam07678
Location:978 → 1311

A2M_comp; A-macroglobulin complement component

pfam17791
Location:233 → 318

MG3; Macroglobulin domain MG3

pfam01835
Location:139 → 231

A2M_N; MG2 domain

pfam07703
Location:470 → 609

A2M_N_2; Alpha-2-macroglobulin family N-terminal region

cl02512
Location:1562 → 1716

NTR_like; NTR_like domain; a beta barrel with an oligosaccharide/oligonucleotide-binding fold found in netrins, complement proteins, tissue inhibitors of metalloproteases (TIMP), and procollagen C-proteinase enhancers (PCOLCE), amongst others. In netrins, the ...