B-cell lymphoma/leukemia 10 - Homo sapiens (Human)

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Reviewed, UniProtKB/Swiss-Prot O95999 (BCL10_HUMAN)

Last modified June 16, 2009. Version 72. History...

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Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Binary interactions · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents

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Names and origin

Protein names

Recommended name:
    B-cell lymphoma/leukemia 10
Alternative name(s):
    B-cell CLL/lymphoma 10
      Short name=Bcl-10
    CED-3/ICH-1 prodomain homologous E10-like regulator
      Short name=CIPER
    CARD-containing molecule enhancing NF-kappa-B
    Cellular homolog of vCARMEN
      Short name=cCARMEN
    Mammalian CARD-containing adapter molecule E10
      Short name=mE10
    Cellular-E10
      Short name=c-E10
    CARD-like apoptotic protein
      Short name=hCLAP

Gene names

Name:	BCL10
Synonyms:	CIPER, CLAP

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

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Protein attributes

Sequence length	233 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is not processed.
Protein existence	Evidence at protein level.

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General annotation (Comments)

Function	Promotes apoptosis, pro-caspase-9 maturation and activation of NF-kappa-B via NIK and IKK. May be an adapter protein between upstream TNFR1-TRADD-RIP complex and the downstream NIK-IKK-IKAP complex. Is a substrate for MALT1. Ref.10
Subunit structure	Self-associates by CARD-CARD interaction and forms a tight complex with MALT1. Interacts with other CARD-proteins such as CARD9, CARD10, CARD11 and CARD14. Binds caspase-9 with its C-terminal domain. Interacts with TRAF2 and BIRC2/c-IAP2.
Subcellular location	Cytoplasm › perinuclear region. Note: Appears to have a perinuclear, compact and filamentous pattern of expression. Also found in the nucleus of several types of tumor cells.
Tissue specificity	Ubiquitous.
Post-translational modification	Phosphorylated. Phosphorylation results in dissociation from TRAF2 and binding to BIRC2/c-IAP2. Ref.9
Involvement in disease	A chromosomal aberration involving BCL10 is recurrent in low-grade mucosa-associated lymphoid tissue (MALT lymphoma). Translocation t(1;14)(p22;q32). Although the BCL10/IgH translocation leaves the coding region of BCL10 intact, frequent BCL10 mutations could be attributed to the Ig somatic hypermutation mechanism resulting in nucleotide transitions. Defects in BCL10 are involved in various types of cancer.
Sequence similarities	Contains 1 CARD domain.

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Ontologies

Keywords
Biological process	Apoptosis Cell cycle
Cellular component	Cytoplasm
Coding sequence diversity	Chromosomal rearrangement
Disease	Disease mutation
Molecular function	Anti-oncogene
PTM	Phosphoprotein
Gene Ontology (GO)
Biological process	T cell receptor signaling pathway Inferred from direct assay. Source: UniProtKB apoptosis Ref.9 Inferred from direct assay. Source: UniProtKB cell cycle Inferred from electronic annotation. Source: UniProtKB-KW innate immune response Inferred from expression pattern. Source: UniProtKB interleukin-6 biosynthetic process Non-traceable author statement. Source: UniProtKB lymphotoxin A biosynthetic process Non-traceable author statement. Source: UniProtKB negative regulation of cell cycle Inferred from electronic annotation. Source: UniProtKB-KW negative regulation of mature B cell apoptosis Inferred from direct assay. Source: UniProtKB neural tube closure Inferred from sequence or structural similarity. Source: UniProtKB positive regulation of I-kappaB kinase/NF-kappaB cascade Inferred from expression pattern. Source: UniProtKB positive regulation of NF-kappaB transcription factor activity Ref.6 Inferred from direct assay. Source: UniProtKB positive regulation of interleukin-8 biosynthetic process Inferred from mutant phenotype. Source: UniProtKB positive regulation of mast cell cytokine production Non-traceable author statement. Source: UniProtKB positive regulation of phosphorylation Inferred from direct assay. Source: UniProtKB positive regulation of protein ubiquitination Inferred from direct assay. Source: UniProtKB positive regulation of transcription, DNA-dependent Inferred from direct assay. Source: UniProtKB protein homooligomerization Traceable author statement. Source: UniProtKB response to molecule of bacterial origin Inferred from expression pattern. Source: UniProtKB toll-like receptor signaling pathway Inferred by curator. Source: UniProtKB
Cellular component	CBM complex Non-traceable author statement. Source: UniProtKB cytosol Inferred from direct assay. Source: UniProtKB lysosome Inferred from direct assay. Source: UniProtKB nucleus Inferred from direct assay. Source: UniProtKB perinuclear region of cytoplasm Inferred from direct assay. Source: UniProtKB
Molecular function	NF-kappaB binding Inferred from direct assay. Source: UniProtKB protein C-terminus binding Inferred from physical interaction. Source: UniProtKB protein kinase B binding Inferred from physical interaction. Source: UniProtKB protein self-association Inferred from physical interaction. Source: UniProtKB transcription coactivator activity Inferred from direct assay. Source: UniProtKB ubiquitin binding Inferred from direct assay. Source: UniProtKB ubiquitin protein ligase binding Inferred from physical interaction. Source: UniProtKB
Complete GO annotation...

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Binary interactions

With	Entry	#Exp.	IntAct
AKT1	P31749	3	EBI-958922,EBI-296087
BCL3	P20749	2	EBI-958922,EBI-958997
HIST1H2BC	P62807	1	EBI-958922,EBI-354552
KRT33B	Q14525	1	EBI-958922,EBI-1049638

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Sequence annotation (Features)

Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Molecule processing

Chain

1 – 233

233

B-cell lymphoma/leukemia 10

PRO_0000144074

Regions

Domain

13 – 101

CARD

Natural variations

Natural variant

A → S in MALT lymphoma and mesothelioma. dbSNP rs12037217.

VAR_013208

Natural variant

V → E in MALT lymphoma.

VAR_013209

Natural variant

K → E in MALT lymphoma.

VAR_013210

Natural variant

K → Q in mesothelioma. Ref.12

VAR_013211

Natural variant

T → I in mesothelioma. Ref.1

VAR_013212

Natural variant

C → R in MALT lymphoma.

VAR_013213

Natural variant

R → G in germ cell tumor. Ref.12 Ref.1

VAR_013214

Natural variant

R → Q in mesothelioma. Ref.12 Ref.1

VAR_013215

Natural variant

R → K in MALT lymphoma.

VAR_013216

Natural variant

N → S in mesothelioma. Ref.12

VAR_013217

Natural variant

101

D → E in MALT lymphoma.

VAR_013218

Natural variant

134

S → P in MALT lymphoma.

VAR_013219

Natural variant

153

M → V in mesothelioma. Ref.12

VAR_013220

Natural variant

168

T → A in MALT lymphoma.

VAR_013221

Natural variant

174

L → S in MALT lymphoma.

VAR_013222

Natural variant

210

Missing in follicular lymphoma. Ref.1

VAR_013223

Natural variant

213

G → E in MALT lymphoma and mesothelioma. dbSNP rs3768235.

VAR_013224

Natural variant

218

S → F in germ cell tumor, mesothelioma and other cancer cell lines. Ref.12 Ref.1

VAR_013225

Natural variant

230

V → I in MALT lymphoma.

VAR_013226

Experimental info

Mutagenesis

L → A: Abolishes cell death-inducing capability. Ref.4

Mutagenesis

L → A: Abolishes cell death-inducing capability. Ref.4 Ref.2

Mutagenesis

L → Q: Abolishes NF-kappa-B activation and homo/hetero-dimerization. Ref.4 Ref.2

Mutagenesis

I → A: Abolishes cell death-inducing capability. Ref.4

Mutagenesis

L → A: Abolishes cell death-inducing capability. Ref.4

Mutagenesis

E → A: Abolishes cell death-inducing capability. Ref.4

Mutagenesis

I → A: Abolishes cell death-inducing capability. Ref.4

Mutagenesis

G → R: Abolishes NF-kappa-B activation. Ref.2

Mutagenesis

228

R → G: Abolishes MALT1-mediated cleavage. Ref.10

Mutagenesis

231

S → A: Promotes NF-kappa-B activation.

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Sequences

Sequence

Length

Mass (Da)

Tools

O95999-1 [UniParc].

Last modified May 1, 1999. Version 1.
Checksum: F87C97F2B784BA4B
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FASTA

233

26,252

        10         20         30         40         50         60 
MEPTAPSLTE EDLTEVKKDA LENLRVYLCE KIIAERHFDH LRAKKILSRE DTEEISCRTS 

        70         80         90        100        110        120 
SRKRAGKLLD YLQENPKGLD TLVESIRREK TQNFLIQKIT DEVLKLRNIK LEHLKGLKCS 

       130        140        150        160        170        180 
SCEPFPDGAT NNLSRSNSDE SNFSEKLRAS TVMYHPEGES STTPFFSTNS SLNLPVLEVG 

       190        200        210        220        230 
RTENTIFSST TLPRPGDPGA PPLPPDLQLE EEGTCANSSE MFLPLRSRTV SRQ

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References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Bcl10 is involved in t(1;14)(p22;q32) of MALT B cell lymphoma and mutated in multiple tumor types." Willis T.G., Jadayel D.M., Du M.-Q., Peng H., Perry A.R., Abdul-Rauf M., Price H., Karran L., Majekodunmi O., Wlodarska I., Pan L., Crook T., Hamoudi R., Isaacson P., Dyer M.J.S. Cell 96:35-45(1999) [PubMed: 9989495] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT FOLLICULAR LYMPHOMA GLU-210 DEL, VARIANT MESOTHELIOMA ILE-52, VARIANTS GERM CELL TUMOR GLY-58 AND PHE-218. Tissue: Lymphoma.
[2]	"CIPER, a novel NF kappaB-activating protein containing a caspase recruitment domain with homology to Herpesvirus-2 protein E10." Koseki T., Inohara N., Chen S., Carrio R., Merino J., Hottiger M.O., Nabel G.J., Nunez G. J. Biol. Chem. 274:9955-9961(1999) [PubMed: 10187770] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], MUTAGENESIS OF LEU-41 AND GLY-78.
[3]	"Equine herpesvirus-2 E10 gene product, but not its cellular homologue, activates NF-kappaB transcription factor and c-Jun N-terminal kinase." Thome M., Martinon F., Hofmann K., Rubio V., Steiner V., Schneider P., Mattmann C., Tschopp J. J. Biol. Chem. 274:9962-9968(1999) [PubMed: 10187771] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[4]	"mE10, a novel caspase recruitment domain-containing proapoptotic molecule." Yan M., Lee J., Schilbach S., Goddard A., Dixit V.M. J. Biol. Chem. 274:10287-10292(1999) [PubMed: 10187815] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], MUTAGENESIS OF LEU-28; LEU-41; ILE-46; LEU-47; GLU-53 AND ILE-55.
[5]	"CLAP, a novel caspase recruitment domain-containing protein in the tumor necrosis factor receptor pathway, regulates NF-kappaB activation and apoptosis." Srinivasula S.M., Ahmad M., Lin J.-H., Poyet J.-L., Fernandes-Alnemri T., Tsichlis P.N., Alnemri E.S. J. Biol. Chem. 274:17946-17954(1999) [PubMed: 10364242] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[6]	"c-E10 is a caspase-recruiting domain-containing protein that interacts with components of death receptors signaling pathway and activates nuclear factor-kappaB." Costanzo A., Guiet C., Vito P. J. Biol. Chem. 274:20127-20132(1999) [PubMed: 10400625] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA]. Tissue: Spleen.
[7]	"Inactivating mutations and overexpression of BCL10, a caspase recruitment domain-containing gene, in MALT lymphoma with t(1;14)(p22;q32)." Zhang Q., Siebert R., Yan M., Hinzmann B., Cui X., Xue L., Rakestraw K.M., Naeve C.W., Beckmann G., Weisenburger D.D., Sanger W.G., Nowotny H., Vesely M., Callet-Bauchu E., Salles G., Dixit V.M., Rosenthal A., Schlegelberger B., Morris S.W. Nat. Genet. 22:63-68(1999) [PubMed: 10319863] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA], VARIANTS MALT LYMPHOMA SER-5; GLU-16; GLU-31; ARG-57; LYS-64; GLU-101; PRO-134; ALA-168; SER-174; GLU-213 AND ILE-230.
[8]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Eye.
[9]	"Interchangeable binding of Bcl10 to TRAF2 and cIAPs regulates apoptosis signaling." Yui D., Yoneda T., Oono K., Katayama T., Imaizumi K., Tohyama M. Oncogene 20:4317-4323(2001) [PubMed: 11466612] [Abstract] Cited for: PHOSPHORYLATION.
[10]	"The proteolytic activity of the paracaspase MALT1 is key in T cell activation." Rebeaud F., Hailfinger S., Posevitz-Fejfar A., Tapernoux M., Moser R., Rueda D., Gaide O., Guzzardi M., Iancu E.M., Rufer N., Fasel N., Thome M. Nat. Immunol. 9:272-281(2008) [PubMed: 18264101] [Abstract] Cited for: FUNCTION, MUTAGENESIS OF ARG-228.
[11]	Colinge J., Superti-Furga G., Bennett K.L. Submitted (OCT-2008) to UniProtKB Cited for: IDENTIFICATION [LARGE SCALE ANALYSIS], MASS SPECTROMETRY.
[12]	"Absence of BCL10 mutations in human malignant mesothelioma." Apostolou S., de Rienzo A., Murthy S.S., Jhanwar S.C., Testa J.R. Cell 97:684-686(1999) [PubMed: 10380921] [Abstract] Cited for: VARIANTS MESOTHELIOMA SER-5; GLN-45; GLN-58; SER-93; VAL-153; GLU-213 AND PHE-218.
+	Additional computationally mapped references.

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Cross-references

Sequence databases
	AJ006288 mRNA. Translation: CAA06955.1. AF057700 mRNA. Translation: AAD15800.1. AF100338 mRNA. Translation: AAD16428.1. AF127386 mRNA. Translation: AAD32597.1. AF134395 mRNA. Translation: AAD39147.1. AF105066 mRNA. Translation: AAF06894.1. AF082283 mRNA. Translation: AAC99767.1. AF097732 Genomic DNA. Translation: AAD24918.1. BC053617 mRNA. Translation: AAH53617.1.
IPI	IPI00022477.
RefSeq	NP_003912.1.
UniGene	Hs.193516
3D structure databases
ModBase	Search...
Protein-protein interaction databases
IntAct	O95999. 4 interactions.
PTM databases
PhosphoSite	O95999.
Proteomic databases
PRIDE	O95999.
Genome annotation databases
Ensembl	ENSG00000142867. Homo sapiens. [Contig view]
GeneID	8915.
KEGG	hsa:8915.
Organism-specific databases
GeneCards	GC01M085443.
H-InvDB	HIX0000744.
HGNC	HGNC:989. BCL10.
HPA	CAB001944.
MIM	603517. gene.
Orphanet	52417. MALT lymphoma.
PharmGKB	PA25299.
GenAtlas	Search...
Phylogenomic databases
HOGENOM	O95999.
HOVERGEN	O95999.
OMA	O95999. STVIYHP.
Enzyme and pathway databases
Pathway_Interaction_DB	bcr_5pathway. BCR signaling pathway. nfkappabcanonicalpathway. Canonical NF-kappaB pathway. tcrpathway. TCR signaling in naive CD4+ T cells. cd8tcrpathway. TCR signaling in naive CD8+ T cells.
Reactome	REACT_6900. Signaling in Immune system.
Gene expression databases
ArrayExpress	O95999.
Bgee	O95999.
CleanEx	HS_BCL10.
GermOnline	ENSG00000142867. Homo sapiens.
Family and domain databases
InterPro	IPR001315. CARD. IPR011029. DEATH-like. [Graphical view]
Gene3D	G3DSA:1.10.533.10. DEATH_like. 1 hit.
Pfam	PF00619. CARD. 1 hit. [Graphical view]
PROSITE	PS50209. CARD. 1 hit. [Graphical view]
ProtoNet	Search...
Other Resources
NextBio	33536.
SOURCE	Search...

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Entry information

Entry name

BCL10_HUMAN

Accession

Primary (citable) accession number: O95999

Entry history

Integrated into UniProtKB/Swiss-Prot:	May 2, 2002
Last sequence update:	May 1, 1999
Last modified:	June 16, 2009
This is version 72 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)

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Relevant documents

Human chromosome 1

Human chromosome 1: entries, gene names and cross-references to MIM

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

SIMILARITY comments

Index of protein domains and families

Names and origin

Protein attributes

General annotation (Comments)

Ontologies

Binary interactions

With

Entry

#Exp.

IntAct

Notes

Sequence annotation (Features)

Molecule processing

Regions

Natural variations

Experimental info

Sequences

References

Cross-references

Sequence databases

3D structure databases

Protein-protein interaction databases

PTM databases

Proteomic databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Gene expression databases

Family and domain databases

Other Resources

Entry information

Relevant documents