P05160 (F13B_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Coagulation factor XIII B chain

Alternative name(s):
Fibrin-stabilizing factor B subunit
Protein-glutamine gamma-glutamyltransferase B chain
Transglutaminase B chain

Gene names

Name:

F13B

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	661 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	The B chain of factor XIII is not catalytically active, but is thought to stabilize the A subunits and regulate the rate of transglutaminase formation by thrombin.
Subunit structure	Tetramer of two A chains and two B chains.
Subcellular location	Secreted.
Involvement in disease	Factor XIII subunit B deficiency (FA13BD) [MIM:613235]: An autosomal recessive hematologic disorder characterized by a life-long bleeding tendency, impaired wound healing and spontaneous abortion in affected women. Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.8 Ref.11
Sequence similarities	Contains 10 Sushi (CCP/SCR) domains.

Ontologies

Keywords
Biological process	Blood coagulation Hemostasis
Cellular component	Secreted
Coding sequence diversity	Polymorphism
Disease	Disease mutation
Domain	Repeat Signal Sushi
PTM	Disulfide bond Glycoprotein
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	blood coagulation Traceable author statement. Source: Reactome
Cellular_component	extracellular region Non-traceable author statement PubMed 14718574. Source: UniProtKB
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Signal peptide	1 – 20	20
	Chain	21 – 661	641	Coagulation factor XIII B chain		PRO_0000021222
Regions
	Domain	24 – 88	65	Sushi 1
	Domain	89 – 148	60	Sushi 2
	Domain	151 – 210	60	Sushi 3
	Domain	211 – 269	59	Sushi 4
	Domain	272 – 329	58	Sushi 5
	Domain	334 – 391	58	Sushi 6
	Domain	394 – 452	59	Sushi 7
	Domain	453 – 516	64	Sushi 8
	Domain	522 – 580	59	Sushi 9
	Domain	581 – 647	67	Sushi 10
	Motif	617 – 619	3	Cell attachment site
Amino acid modifications
	Glycosylation	162	1	N-linked (GlcNAc...) Ref.10
	Glycosylation	545	1	N-linked (GlcNAc...) Ref.9
	Disulfide bond	25 ↔ 76		By similarity
	Disulfide bond	59 ↔ 87		By similarity
	Disulfide bond	91 ↔ 135		By similarity
	Disulfide bond	118 ↔ 146		By similarity
	Disulfide bond	153 ↔ 197		By similarity
	Disulfide bond	180 ↔ 208		By similarity
	Disulfide bond	213 ↔ 255		By similarity
	Disulfide bond	241 ↔ 267		By similarity
	Disulfide bond	274 ↔ 316		By similarity
	Disulfide bond	302 ↔ 327		By similarity
	Disulfide bond	336 ↔ 378		By similarity
	Disulfide bond	364 ↔ 389		By similarity
	Disulfide bond	396 ↔ 439		By similarity
	Disulfide bond	425 ↔ 450		By similarity
	Disulfide bond	454 ↔ 505		By similarity
	Disulfide bond	486 ↔ 515		By similarity
	Disulfide bond	524 ↔ 567		By similarity
	Disulfide bond	553 ↔ 578		By similarity
	Disulfide bond	582 ↔ 636		By similarity
	Disulfide bond	616 ↔ 646		By similarity
Natural variations
	Natural variant	49	1	M → V. Ref.12 Corresponds to variant rs6002 [ dbSNP | Ensembl ].		VAR_013930
	Natural variant	115	1	R → H. Ref.1 Ref.2 Ref.5 Ref.12 Corresponds to variant rs6003 [ dbSNP | Ensembl ].		VAR_013931
	Natural variant	342	1	I → T. Ref.5 Corresponds to variant rs17514281 [ dbSNP | Ensembl ].		VAR_020612
	Natural variant	350	1	H → R. Ref.5 Ref.12 Corresponds to variant rs5999 [ dbSNP | Ensembl ].		VAR_013932
	Natural variant	388	1	E → V. Ref.12 Corresponds to variant rs5991 [ dbSNP | Ensembl ].		VAR_013933
	Natural variant	450	1	C → F in FA13BD. Ref.11		VAR_007475
	Natural variant	529	1	L → P. Ref.5 Corresponds to variant rs17549671 [ dbSNP | Ensembl ].		VAR_020613
	Natural variant	543	1	Y → S. Ref.12 Corresponds to variant rs6001 [ dbSNP | Ensembl ].		VAR_013934
	Natural variant	569	1	D → E. Ref.4 Ref.5 Ref.12 Corresponds to variant rs6000 [ dbSNP | Ensembl ].		VAR_013935

Sequences

	Sequence		Length	Mass (Da)
	P05160 [UniParc]. Last modified April 17, 2007. Version 3. Checksum: 57A2FD2A0E35B812	FASTA	661	75,511
	10 20 30 40 50 60 MRLKNLTFII ILIISGELYA EEKPCGFPHV ENGRIAQYYY TFKSFYFPMS IDKKLSFFCL 70 80 90 100 110 120 AGYTTESGRQ EEQTTCTTEG WSPEPRCFKK CTKPDLSNGY ISDVKLLYKI QENMRYGCAS 130 140 150 160 170 180 GYKTTGGKDE EVVQCLSDGW SSQPTCRKEH ETCLAPELYN GNYSTTQKTF KVKDKVQYEC 190 200 210 220 230 240 ATGYYTAGGK KTEEVECLTY GWSLTPKCTK LKCSSLRLIE NGYFHPVKQT YEEGDVVQFF 250 260 270 280 290 300 CHENYYLSGS DLIQCYNFGW YPESPVCEGR RNRCPPPPLP INSKIQTHST TYRHGEIVHI 310 320 330 340 350 360 ECELNFEIHG SAEIRCEDGK WTEPPKCIEG QEKVACEEPP FIENGAANLH SKIYYNGDKV 370 380 390 400 410 420 TYACKSGYLL HGSNEITCNR GKWTLPPECV ENNENCKHPP VVMNGAVADG ILASYATGSS 430 440 450 460 470 480 VEYRCNEYYL LRGSKISRCE QGKWSSPPVC LEPCTVNVDY MNRNNIEMKW KYEGKVLHGD 490 500 510 520 530 540 LIDFVCKQGY DLSPLTPLSE LSVQCNRGEV KYPLCTRKES KGMCTSPPLI KHGVIISSTV 550 560 570 580 590 600 DTYENGSSVE YRCFDHHFLE GSREAYCLDG MWTTPPLCLE PCTLSFTEME KNNLLLKWDF 610 620 630 640 650 660 DNRPHILHGE YIEFICRGDT YPAELYITGS ILRMQCDRGQ LKYPRCIPRQ STLSYQEPLR T

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Nucleotide sequence of the gene for the b subunit of human factor XIII." Bottenus R.E., Ichinose A., Davie E.W. Biochemistry 29:11195-11209(1990) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT HIS-115.
[2]	"Amino acid sequence of the b subunit of human factor XIII, a protein composed of ten repetitive segments." Ichinose A., McMullen B.A., Fujikawa K., Davie E.W. Biochemistry 25:4633-4638(1986) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 2-661, VARIANT HIS-115.
[3]	Ichinose A. Submitted (FEB-1987) to the EMBL/GenBank/DDBJ databases Cited for: SEQUENCE REVISION.
[4]	"Complete sequencing and characterization of 21,243 full-length human cDNAs." Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S. Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANT GLU-569. Tissue: Heart.
[5]	SeattleSNPs variation discovery resource Submitted (JUL-2004) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANTS HIS-115; THR-342; ARG-350; PRO-529 AND GLU-569.
[6]	"The DNA sequence and biological annotation of human chromosome 1." Gregory S.G., Barlow K.F., McLay K.E., Kaul R., Swarbreck D., Dunham A., Scott C.E., Howe K.L., Woodfine K., Spencer C.C.A., Jones M.C., Gillson C., Searle S., Zhou Y., Kokocinski F., McDonald L., Evans R., Phillips K. , Atkinson A., Cooper R., Jones C., Hall R.E., Andrews T.D., Lloyd C., Ainscough R., Almeida J.P., Ambrose K.D., Anderson F., Andrew R.W., Ashwell R.I.S., Aubin K., Babbage A.K., Bagguley C.L., Bailey J., Beasley H., Bethel G., Bird C.P., Bray-Allen S., Brown J.Y., Brown A.J., Buckley D., Burton J., Bye J., Carder C., Chapman J.C., Clark S.Y., Clarke G., Clee C., Cobley V., Collier R.E., Corby N., Coville G.J., Davies J., Deadman R., Dunn M., Earthrowl M., Ellington A.G., Errington H., Frankish A., Frankland J., French L., Garner P., Garnett J., Gay L., Ghori M.R.J., Gibson R., Gilby L.M., Gillett W., Glithero R.J., Grafham D.V., Griffiths C., Griffiths-Jones S., Grocock R., Hammond S., Harrison E.S.I., Hart E., Haugen E., Heath P.D., Holmes S., Holt K., Howden P.J., Hunt A.R., Hunt S.E., Hunter G., Isherwood J., James R., Johnson C., Johnson D., Joy A., Kay M., Kershaw J.K., Kibukawa M., Kimberley A.M., King A., Knights A.J., Lad H., Laird G., Lawlor S., Leongamornlert D.A., Lloyd D.M., Loveland J., Lovell J., Lush M.J., Lyne R., Martin S., Mashreghi-Mohammadi M., Matthews L., Matthews N.S.W., McLaren S., Milne S., Mistry S., Moore M.J.F., Nickerson T., O'Dell C.N., Oliver K., Palmeiri A., Palmer S.A., Parker A., Patel D., Pearce A.V., Peck A.I., Pelan S., Phelps K., Phillimore B.J., Plumb R., Rajan J., Raymond C., Rouse G., Saenphimmachak C., Sehra H.K., Sheridan E., Shownkeen R., Sims S., Skuce C.D., Smith M., Steward C., Subramanian S., Sycamore N., Tracey A., Tromans A., Van Helmond Z., Wall M., Wallis J.M., White S., Whitehead S.L., Wilkinson J.E., Willey D.L., Williams H., Wilming L., Wray P.W., Wu Z., Coulson A., Vaudin M., Sulston J.E., Durbin R.M., Hubbard T., Wooster R., Dunham I., Carter N.P., McVean G., Ross M.T., Harrow J., Olson M.V., Beck S., Rogers J., Bentley D.R. Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[7]	"Complete cDNA sequence encoding the B subunit of human factor XIII." Grundmann U., Nerlich C., Rein T., Zettlmeissl G. Nucleic Acids Res. 18:2817-2817(1990) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 1-20. Tissue: Liver.
[8]	"Truncated mutant B subunit for factor XIII causes its deficiency due to impaired intracellular transportation." Koseki S., Souri M., Koga S., Yamakawa M., Shichishima T., Maruyama Y., Yanai F., Ichinose A. Blood 97:2667-2672(2001) [PubMed] [Europe PMC] [Abstract] Cited for: INVOLVEMENT IN FA13BD.
[9]	"Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry." Liu T., Qian W.-J., Gritsenko M.A., Camp D.G. II, Monroe M.E., Moore R.J., Smith R.D. J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-545, MASS SPECTROMETRY. Tissue: Plasma.
[10]	"Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry." Chen R., Jiang X., Sun D., Han G., Wang F., Ye M., Wang L., Zou H. J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-162, MASS SPECTROMETRY. Tissue: Liver.
[11]	"Two genetic defects in a patient with complete deficiency of the b-subunit for coagulation factor XIII." Hashiguchi T., Saito M., Morishita E., Matsuda T., Ichinose A. Blood 82:145-150(1993) [PubMed] [Europe PMC] [Abstract] Cited for: VARIANT FA13BD PHE-450.
[12]	"Characterization of single-nucleotide polymorphisms in coding regions of human genes." Cargill M., Altshuler D., Ireland J., Sklar P., Ardlie K., Patil N., Shaw N., Lane C.R., Lim E.P., Kalyanaraman N., Nemesh J., Ziaugra L., Friedland L., Rolfe A., Warrington J., Lipshutz R., Daley G.Q., Lander E.S. Nat. Genet. 22:231-238(1999) [PubMed] [Europe PMC] [Abstract] Cited for: VARIANTS VAL-49; HIS-115; ARG-350; VAL-388; SER-543 AND GLU-569.
[13]	Erratum Cargill M., Altshuler D., Ireland J., Sklar P., Ardlie K., Patil N., Shaw N., Lane C.R., Lim E.P., Kalyanaraman N., Nemesh J., Ziaugra L., Friedland L., Rolfe A., Warrington J., Lipshutz R., Daley G.Q., Lander E.S. Nat. Genet. 23:373-373(1999)
+	Additional computationally mapped references.

Web resources

SHMPD

The Singapore human mutation and polymorphism database

Wikipedia

Factor XIII entry

SeattleSNPs

Cross-references

Sequence databases
EMBL GenBank DDBJ	M64554 Genomic DNA. Translation: AAA51821.1. Sequence problems. M14057 mRNA. Translation: AAA88042.1. AK290560 mRNA. Translation: BAF83249.1. AY692223 Genomic DNA. Translation: AAT85802.1. AL353809 Genomic DNA. Translation: CAH72549.1. X51823 mRNA. Translation: CAA36123.1.
IPI	IPI00007240.
PIR	KFHU13. A36397.
RefSeq	NP_001985.2. NM_001994.2.
UniGene	Hs.435782.
3D structure databases
ProteinModelPortal	P05160.
ModBase	Search...
Protein-protein interaction databases
STRING	9606.ENSP00000356382.
PTM databases
PhosphoSite	P05160.
Polymorphism databases
DMDM	145559473.
Proteomic databases
PaxDb	P05160.
PeptideAtlas	P05160.
PRIDE	P05160.
Protocols and materials databases
DNASU	2165.
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000367412; ENSP00000356382; ENSG00000143278.
GeneID	2165.
KEGG	hsa:2165.
UCSC	uc001gtt.1. human.
Organism-specific databases
CTD	2165.
GeneCards	GC01M197008.
H-InvDB	HIX0028859.
HGNC	HGNC:3534. F13B.
HPA	HPA003827.
MIM	134580. gene+phenotype. 613235. phenotype.
neXtProt	NX_P05160.
Orphanet	331. Congenital factor XIII deficiency.
PharmGKB	PA27944.
GenAtlas	Search...
Phylogenomic databases
eggNOG	NOG146710.
HOGENOM	HOG000049250.
HOVERGEN	HBG005626.
InParanoid	P05160.
KO	K03906.
OMA	CELNFEI.
OrthoDB	EOG4001HQ.
PhylomeDB	P05160.
Enzyme and pathway databases
Reactome	REACT_604. Hemostasis.
Gene expression databases
Bgee	P05160.
CleanEx	HS_F13B.
Genevestigator	P05160.
GermOnline	ENSG00000143278. Homo sapiens.
Family and domain databases
InterPro	IPR000436. Sushi_SCR_CCP. [Graphical view]
Pfam	PF00084. Sushi. 8 hits. [Graphical view]
SMART	SM00032. CCP. 8 hits. [Graphical view]
SUPFAM	SSF57535. Complement_control_module. 10 hits.
PROSITE	PS50923. SUSHI. 7 hits. [Graphical view]
ProtoNet	Search...
Other
GenomeRNAi	2165.
NextBio	8743.
SOURCE	Search...

Entry information

Entry name

F13B_HUMAN

Accession

Primary (citable) accession number: P05160
Secondary accession number(s): A8K3E5, Q5VYL5

Entry history

Integrated into UniProtKB/Swiss-Prot:	August 13, 1987
Last sequence update:	April 17, 2007
Last modified:	May 1, 2013
This is version 138 of the entry and version 3 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.