Reviewed, UniProtKB/Swiss-Prot Q60824 (BPAEB_MOUSE)

Names and origin

Protein names

Recommended name:
    Bullous pemphigoid antigen 1, isoforms 6/7
      Short name=BPA
Alternative name(s):
    Hemidesmosomal plaque protein
    Dystonia musculorum protein
      Short name=Dystonin

Gene names

Name:	Dst
Synonyms:	Bpag1

Organism

Mus musculus (Mouse)

Taxonomic identifier

10090 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Mus

Protein attributes

Sequence length	1678 AA.
Sequence status	Fragments.
Sequence processing	The displayed sequence is not processed.
Protein existence	Evidence at protein level.

General annotation (Comments)

Function	Cytoskeletal linker protein. Anchors keratin-containing intermediate filaments to the inner plaque of hemidesmosomes. The proteins may self-aggregate to form filaments or a two-dimensional mesh. Ref.2
Subunit structure	Homodimer. Interacts with the neuronal intermediate filament protein, Prph. Ref.2
Subcellular location	Cytoplasm › cytoskeleton By similarity.
Sequence similarities	Belongs to the plakin or cytolinker family. UniProtKB Q03001 Contains 1 actin-binding domain. Contains 2 CH (calponin-homology) domains. Contains 11 plectin repeats.

Ontologies

Keywords
Biological process	Cell adhesion
Cellular component	Cytoplasm Cytoskeleton
Coding sequence diversity	Alternative splicing
Domain	Coiled coil Repeat
Ligand	Actin-binding
Gene Ontology (GO)
Biological process	cell adhesion Inferred from electronic annotation. Source: UniProtKB-KW intermediate filament cytoskeleton organization Inferred from sequence or structural similarity. Source: UniProtKB
Cellular component	cytoplasm Inferred from sequence or structural similarity. Source: UniProtKB cytoskeleton Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	actin binding Inferred from electronic annotation. Source: UniProtKB-KW
Complete GO annotation...

Binary interactions

With	Entry	#Exp.	IntAct	Notes
Prph	P21807	1	EBI-446159,EBI-446227	From a different organism.

Alternative products

This entry describes 7 isoforms produced by alternative splicing. [Align] [Select]

Isoform 7 Ref.1 (identifier: Q60824-2)

Also known as: n2;

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

Isoform 1 Ref.3 (identifier: Q91ZU6-2)

Also known as: a;

The sequence of this isoform can be found in the external entry Q91ZU6-2.
Isoforms of the same protein are often annotated in two different entries if their sequences differ significantly.

Isoform 2 Ref.3 (identifier: Q91ZU6-1)

Also known as: b;

The sequence of this isoform can be found in the external entry Q91ZU6-1.
Isoforms of the same protein are often annotated in two different entries if their sequences differ significantly.

Isoform 3 (identifier: Q91ZU6-3)

The sequence of this isoform can be found in the external entry Q91ZU6-3.
Isoforms of the same protein are often annotated in two different entries if their sequences differ significantly.

Note: No experimental confirmation available.

Isoform 4 (identifier: Q91ZU6-4)

The sequence of this isoform can be found in the external entry Q91ZU6-4.
Isoforms of the same protein are often annotated in two different entries if their sequences differ significantly.

Note: No experimental confirmation available.

Isoform 5 Ref.3 (identifier: Q91ZU8-1)

Also known as: e;

The sequence of this isoform can be found in the external entry Q91ZU8-1.
Isoforms of the same protein are often annotated in two different entries if their sequences differ significantly.

Isoform 6 Ref.1 (identifier: Q60824-1)

Also known as: n1;

The sequence of this isoform differs from the canonical sequence as follows:
     1-175: Missing.
     176-205: WNLPKHERSKRKIQGGSVLDPAERAVLRIA → MAGYLSPAAYMYVEEQEYLQAYEDVLERYK

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	‹1 – 1678	›1678	Bullous pemphigoid antigen 1, isoforms 6/7		PRO_0000078143
Regions
	Domain	206 – 430	225	Actin-binding
	Domain	210 – 313	104	CH 1
	Domain	326 – 427	102	CH 2
	Repeat	1158 – 1195	38	Plectin 1
	Repeat	1196 – 1233	38	Plectin 2
	Repeat	1234 – 1269	36	Plectin 3
	Repeat	1270 – 1307	38	Plectin 4
	Repeat	1349 – 1394	46	Plectin 5
	Repeat	1395 – 1445	51	Plectin 6
	Repeat	1465 – 1504	40	Plectin 7
	Repeat	1505 – 1542	38	Plectin 8
	Repeat	1543 – 1580	38	Plectin 9
	Repeat	1581 – 1618	38	Plectin 10
	Repeat	1619 – 1656	38	Plectin 11
Natural variations
	Alternative sequence	1 – 175	175	Missing in isoform 6. Ref.1		VSP_050481
	Alternative sequence	176 – 205	30	WNLPK…VLRIA → MAGYLSPAAYMYVEEQEYLQ AYEDVLERYK in isoform 6. Ref.1		VSP_050482
Experimental info
	Non-adjacent residues	686 – 687	2
	Non-terminal residue	1	1

Sequences

	Sequence		Length	Mass (Da)
	Isoform 7 (n2) [UniParc]. Last modified April 30, 2003. Version 2. Checksum: FDBBEB1EFFAE760F	FASTA	1,678	191,867
	10 20 30 40 50 60 RRFPRFAETL QHPMCSLPLV APAGHRRHHR LLLLLAPQAP ERETSDEIVF SGRSRSRDAA 70 80 90 100 110 120 LRSHHFRSEG FRASPRHIRR RVAAAAAARL EEVKPVVKVH HQSEQESSGR KRRIKKNSRV 130 140 150 160 170 180 QPEFYHSVQG ASTRRPSSGN ASYRCSMSSS ADFSDEDDFS QKSGSASPAP GDTLPWNLPK 190 200 210 220 230 240 HERSKRKIQG GSVLDPAERA VLRIADERDK VQKKTFTKWI NQHLMKVRKH VNDLYEDLRD 250 260 270 280 290 300 GHNLISLLEV LSGDTLPREK GRMRFHRLQN VQIALDYLKR RQVKLVNIRN DDITDGNPKL 310 320 330 340 350 360 TLGLIWTIIL HFQISDIHVT GESEDMSAKE RLLLWTQQAT EGYAGVRCEN FTTCWRDGKL 370 380 390 400 410 420 FNAIIHKYRP DLIDMNTVAV QSNLANLEHA FYVAEKIGVI RLLDPEDVDV SSPDEKSVIT 430 440 450 460 470 480 YVSSLYDAFP KVPEGGEGIG ANDVEVKWIE YQNMVNYLIQ WIRHHVVTMS ERTFPNNPLE 490 500 510 520 530 540 LKALYNQYLQ FKEKEIPPKE MEKSKIKRLY KLLEIWIEFG RIKLLQGYHP NDIEKEWGKL 550 560 570 580 590 600 IIAMLEREKA LRPEVERLDM LQQIATRVQR DSVSCEDKLI LARNALQSDS KRLESGVQFQ 610 620 630 640 650 660 NEAEIAGYIL ECENLLRQHV IDVQILIDGK YYQADQLVQR VAKLRDEIMA LRNECSSVYS 670 680 690 700 710 720 KGRMLTTEQT KLMISGITQS LNSGFANLVS EFKQKCDQQS MIIQKTEKEV RSLSAELSAS 730 740 750 760 770 780 KEEKRREEQK AQLQRAQVQE LNDRLKRVQD ELHLKTIEEQ MTHRKMILLQ EESDKFKRSA 790 800 810 820 830 840 DEFRKKMEKL MESKVVTETD LSGIKHDFVS LQRENFRAQE NAKLWETNIR ELERQLQCYR 850 860 870 880 890 900 EKMQQGPPVE ANHYQKCRRL EEELLAQRRE VENLKQKMDQ QIKEHEHQLL RLQCEIQKKS 910 920 930 940 950 960 TTQDHTFASA FDTAGRECHH PAEISPGNSG HLNLKTRLPL SRWTQEPHQT EGKWPHRAAE 970 980 990 1000 1010 1020 QLPKEVQFRQ PGAPLDRESS QPCYSEYFSQ TSTELQITFD DKNPITRLSE LETMREQALH 1030 1040 1050 1060 1070 1080 PSRPPVTYQD DKLERELVKL LTPLEIAKNK QCGMHTEVTT LKQEKRLGSS AGGWMLEGCR 1090 1100 1110 1120 1130 1140 TSGGLKGDFL KKSVEPEASP SLDLNQACSV RDEEFQFQGL RHTVTGRQLV EAKLLDMRTV 1150 1160 1170 1180 1190 1200 EQLRLGLKTV EEVQRSLSKF LTKATSIAGL YLESSKEKMS FTSAAQKIII DKMIALAFLE 1210 1220 1230 1240 1250 1260 AQAATGFIID PVSGQTYCVE DAVLHGIVDP EFRSRLLEAE KAVLGYSHAS KTLSVFQAME 1270 1280 1290 1300 1310 1320 NRMLDRKKGK HILEAQIASG GVIDPVRGVR VPPEMAVQQG LLNNAVLQFL HEPSSNTRVF 1330 1340 1350 1360 1370 1380 PNPNNKQALY YSELLQICVF DVDCQCFLLP FGEREISNLN IEKTHKIAVV DTKTGAELTA 1390 1400 1410 1420 1430 1440 FEAFQRNLID KGIYLELSGQ QYQWKEATFF DSYGHPSHML TDTKTGLQFN ISEAVEQGTL 1450 1460 1470 1480 1490 1500 DKALVQKYQE GLTTLTELAD FLLSKVVPKK DLHSPIAGYW LTASGERISL LKASRRNLVD 1510 1520 1530 1540 1550 1560 RVTALRCLEA QICTGGIIDP LTGKKYRVAE ALHRGLVDEG FAQQLRQCEL VITGISHPVS 1570 1580 1590 1600 1610 1620 NKMMSVVEAV NANIISKEMG MRCLEFQYLT GGLIEPKVFS RLTIEEALHV GIIDVLIATR 1630 1640 1650 1660 1670 LKDQKSYVRD IMCPQTKRKL TYKEALEKAD FDFHTGLKLL EVSEPLGTGI SNLYYSSQ
	Isoform 1 (a). See Q91ZU6.	FASTA	–	–
	Isoform 2 (b). See Q91ZU6.	FASTA	–	–
	Isoform 3. See Q91ZU6.	FASTA	–	–
	Isoform 4. See Q91ZU6.	FASTA	–	–
	Isoform 5 (e). See Q91ZU8.	FASTA	–	–
	Isoform 6 (n1). Checksum: BEB839154C4BC703	FASTA	1,503	172,482
	10 20 30 40 50 60 MAGYLSPAAY MYVEEQEYLQ AYEDVLERYK DERDKVQKKT FTKWINQHLM KVRKHVNDLY 70 80 90 100 110 120 EDLRDGHNLI SLLEVLSGDT LPREKGRMRF HRLQNVQIAL DYLKRRQVKL VNIRNDDITD 130 140 150 160 170 180 GNPKLTLGLI WTIILHFQIS DIHVTGESED MSAKERLLLW TQQATEGYAG VRCENFTTCW 190 200 210 220 230 240 RDGKLFNAII HKYRPDLIDM NTVAVQSNLA NLEHAFYVAE KIGVIRLLDP EDVDVSSPDE 250 260 270 280 290 300 KSVITYVSSL YDAFPKVPEG GEGIGANDVE VKWIEYQNMV NYLIQWIRHH VVTMSERTFP 310 320 330 340 350 360 NNPLELKALY NQYLQFKEKE IPPKEMEKSK IKRLYKLLEI WIEFGRIKLL QGYHPNDIEK 370 380 390 400 410 420 EWGKLIIAML EREKALRPEV ERLDMLQQIA TRVQRDSVSC EDKLILARNA LQSDSKRLES 430 440 450 460 470 480 GVQFQNEAEI AGYILECENL LRQHVIDVQI LIDGKYYQAD QLVQRVAKLR DEIMALRNEC 490 500 510 520 530 540 SSVYSKGRML TTEQTKLMIS GITQSLNSGF ANLVSEFKQK CDQQSMIIQK TEKEVRSLSA 550 560 570 580 590 600 ELSASKEEKR REEQKAQLQR AQVQELNDRL KRVQDELHLK TIEEQMTHRK MILLQEESDK 610 620 630 640 650 660 FKRSADEFRK KMEKLMESKV VTETDLSGIK HDFVSLQREN FRAQENAKLW ETNIRELERQ 670 680 690 700 710 720 LQCYREKMQQ GPPVEANHYQ KCRRLEEELL AQRREVENLK QKMDQQIKEH EHQLLRLQCE 730 740 750 760 770 780 IQKKSTTQDH TFASAFDTAG RECHHPAEIS PGNSGHLNLK TRLPLSRWTQ EPHQTEGKWP 790 800 810 820 830 840 HRAAEQLPKE VQFRQPGAPL DRESSQPCYS EYFSQTSTEL QITFDDKNPI TRLSELETMR 850 860 870 880 890 900 EQALHPSRPP VTYQDDKLER ELVKLLTPLE IAKNKQCGMH TEVTTLKQEK RLGSSAGGWM 910 920 930 940 950 960 LEGCRTSGGL KGDFLKKSVE PEASPSLDLN QACSVRDEEF QFQGLRHTVT GRQLVEAKLL 970 980 990 1000 1010 1020 DMRTVEQLRL GLKTVEEVQR SLSKFLTKAT SIAGLYLESS KEKMSFTSAA QKIIIDKMIA 1030 1040 1050 1060 1070 1080 LAFLEAQAAT GFIIDPVSGQ TYCVEDAVLH GIVDPEFRSR LLEAEKAVLG YSHASKTLSV 1090 1100 1110 1120 1130 1140 FQAMENRMLD RKKGKHILEA QIASGGVIDP VRGVRVPPEM AVQQGLLNNA VLQFLHEPSS 1150 1160 1170 1180 1190 1200 NTRVFPNPNN KQALYYSELL QICVFDVDCQ CFLLPFGERE ISNLNIEKTH KIAVVDTKTG 1210 1220 1230 1240 1250 1260 AELTAFEAFQ RNLIDKGIYL ELSGQQYQWK EATFFDSYGH PSHMLTDTKT GLQFNISEAV 1270 1280 1290 1300 1310 1320 EQGTLDKALV QKYQEGLTTL TELADFLLSK VVPKKDLHSP IAGYWLTASG ERISLLKASR 1330 1340 1350 1360 1370 1380 RNLVDRVTAL RCLEAQICTG GIIDPLTGKK YRVAEALHRG LVDEGFAQQL RQCELVITGI 1390 1400 1410 1420 1430 1440 SHPVSNKMMS VVEAVNANII SKEMGMRCLE FQYLTGGLIE PKVFSRLTIE EALHVGIIDV 1450 1460 1470 1480 1490 1500 LIATRLKDQK SYVRDIMCPQ TKRKLTYKEA LEKADFDFHT GLKLLEVSEP LGTGISNLYY SSQ

References

[1]	"The mouse dystonia musculorum gene is a neural isoform of bullous pemphigoid antigen 1." Brown A., Bernier G., Mathieu M., Rossant J., Kothary R. Nat. Genet. 10:301-306(1995) [PubMed: 7670468] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 1-686 (ISOFORMS 6 AND 7). Tissue: Brain.
[2]	"The intermediate filament protein peripherin is the specific interaction partner of mouse BPAG1-n (dystonin) in neurons." Leung C.L., Sun D., Liem R.K. J. Cell Biol. 144:435-446(1999) [PubMed: 9971739] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 687-1678, FUNCTION, INTERACTION WITH PRPH. Strain: BALB/c.
[3]	"The BPAG1 locus: alternative splicing produces multiple isoforms with distinct cytoskeletal linker domains, including predominant isoforms in neurons and muscles." Leung C.L., Zheng M., Prater S.M., Liem R.K.H. J. Cell Biol. 154:691-697(2001) [PubMed: 11514586] [Abstract] Cited for: ALTERNATIVE SPLICING. Strain: BALB/c. Tissue: Muscle.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBLGenBankDDBJ	U25158 mRNA. Translation: AAC52231.1. U22452 mRNA. Translation: AAC52230.1. AF115383 mRNA. Translation: AAD22959.1.
IPI	IPI00277410. IPI00623531.
PIR	I49290. I49298.
RefSeq	NP_598594.2. NP_604443.2.
UniGene	Mm.336625
3D structure databases
SMR	Q60824. Positions 200-433.
ModBase	Search...
Protein-protein interaction databases
IntAct	Q60824. 3 interactions.
STRING	Q60824.
Proteomic databases
PRIDE	Q60824.
Genome annotation databases
Ensembl	ENSMUST00000088270; ENSMUSP00000085605; ENSMUSG00000026131; Mus musculus. [Genome view]
GeneID	13518.
KEGG	mmu:13518.
Organism-specific databases
CTD	13518.
MGI	MGI:104627. Dst.
Phylogenomic databases
HOVERGEN	Q60824.
Gene expression databases
ArrayExpress	Q60824.
Bgee	Q60824.
CleanEx	MM_DST.
Genevestigator	Q60824.
GermOnline	ENSMUSG00000026131. Mus musculus.
Family and domain databases
InterPro	IPR001589. Actinin_actin-bd_CS. IPR016146. Calponin-homology. IPR001715. Calponin_act_bd. IPR001101. Plectin_repeat. [Graphical view]
Gene3D	G3DSA:1.10.418.10. Calponin-homology. 2 hits.
Pfam	PF00307. CH. 2 hits. PF00681. Plectin. 5 hits. [Graphical view]
SMART	SM00033. CH. 2 hits. SM00250. PLEC. 11 hits. [Graphical view]
PROSITE	PS00019. ACTININ_1. 1 hit. PS00020. ACTININ_2. 1 hit. PS50021. CH. 2 hits. PS00018. EF_HAND_1. Partial match. [Graphical view]
ProtoNet	Search...
Other Resources
NextBio	284082.
SOURCE	Search...

Entry information

Entry name

BPAEB_MOUSE

Accession

Primary (citable) accession number: Q60824
Secondary accession number(s): Q60845, Q9WU50

Entry history

Integrated into UniProtKB/Swiss-Prot:	April 30, 2003
Last sequence update:	April 30, 2003
Last modified:	November 24, 2009
This is version 88 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)