PiSITE

PDB ID	1B3U	CHAIN	A
Protein name	PROTEIN (PROTEIN PHOSPHATASE PP2A)
Uniprot Accession	P30153
The number of similar proteins	19
The number of binding states	8
The number of binding partners	7

Coloring

Unicolor (beige)

The number of binding partners

Group

Binding state	Binding partners
	1B3U (CHAIN: A)
1	Monomeric state
2	P67775
3	Q9W9P1
4	P67775 Q13362
5	Q13033 Q13033
6	P67775 Q9Y5P8
7	P67775 P63151
8	P67775 Q8BH58

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Only interaction residues

#binding
partners

	5
	4
	3
	2
	1
	0

Sequence information

AAADGDDSLY

PIAVLIDELR

NEDVQLRLNS

IKKLSTIALA

LGVERTRSEL

LPFLTDTIYD

EDEVLLALAE

QLGTFTTLVG

GPEYVHCLLP

PLESLATVEE

100

101

TVVRDKAVES

LRAISHEHSP

SDLEAHFVPL

VKRLAGGDWF

TSRTSACGLF

150

151

SVCYPRVSSA

VKAELRQYFR

NLCSDDTPMV

RRAAASKLGE

FAKVLELDNV

200

201

KSEIIPMFSN

LASDEQDSVR

LLAVEACVNI

AQLLPQEDLE

ALVMPTLRQA

250

251

AEDKSWRVRY

MVADKFTELQ

KAVGPEITKT

DLVPAFQNLM

KDCEAEVRAA

300

301

ASHKVKEFCE

NLSADCRENV

IMSQILPCIK

ELVSDANQHV

KSALASVIMG

350

351

LSPILGKDNT

IEHLLPLFLA

QLKDECPEVR

LNIISNLDCV

NEVIGIRQLS

400

401

QSLLPAIVEL

AEDAKWRVRL

AIIEYMPLLA

GQLGVEFFDE

KLNSLCMAWL

450

451

VDHVYAIREA

ATSNLKKLVE

KFGKEWAHAT

IIPKVLAMSG

DPNYLHRMTT

500

501

LFCINVLSEV

CGQDITTKHM

LPTVLRMAGD

PVANVRFNVA

KSLQKIGPIL

550

551

DNSTLQSEVK

PILEKLTQDQ

DVDVKYFAQE

ALTVLSLA

600

Variants

Residue	AA	Source / dbSNP	Clinical Significance	Allele Frequency (> 0.0001)	AlphaMissense pathogenicity	Disease name
2_GLY	VAL	8.3kJPN chr11:111637042 -	-	0.0001	-	-
3_GLY	ARG	8.3kJPN chr11:111637040 rs1555053169	-	0.0001	-	-
9_LEU	VAL	8.3kJPN chr19:52693377 rs1298006628	-	0.0001	0.8956	-
25_GLN	ARG	8.3kJPN chr11:111636973 -	-	0.0001	0.8967	-
31_ILE	MET	ClinVar chr19:52705214 -	Likely pathogenic	-	0.9231	Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome [MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284]
58_ILE	VAL	gnomAD chr11:111635624 rs372351210	-	0.000482245	0.2998	-
65_LEU	VAL	8.3kJPN chr11:111635603 rs1555052522	-	0.0001	0.9957	-
76_THR	ALA	8.3kJPN chr19:52709275 -	-	0.0001	0.1748	-
77_GLY	ASP	ClinVar chr11:111635566 rs1805076	Pathogenic	-	-	Lung carcinoma [MONDO:MONDO:0005138,MedGen:C0684249]
77_GLY	ASP	gnomAD chr11:111635566 rs1805076	-	0.00651629	-	-
86_HIS	TYR	gnomAD chr19:52709305 rs144146832	-	0.00193104	0.1151	-
104_ARG	CYS	8.3kJPN chr11:111631733 rs782083920	-	0.0007	0.9998	-
106_LYS	ARG	8.3kJPN chr19:52714562 -	-	0.0001	0.1664	-
117_GLU	ASP	8.3kJPN chr11:111631692 -	-	0.0003	0.138	-
127_PHE	LEU	8.3kJPN chr19:52714624 -	-	0.0001	0.9794	-
131_VAL	LEU	VAR_073718 -	LP/P	-	0.3455	Intellectual developmental disorder, autosomal dominant 36 (MRD36) [MIM:616362]
134_LEU	SER	8.3kJPN chr11:111631642 -	-	0.0001	0.9996	-
138_ASP	GLU	gnomAD chr11:111631629 rs545584093	-	0.000107381	0.843	-
144_THR	LYS	gnomAD chr11:111631612 rs115633648	-	0.000998314	0.9987	-
144_THR	LYS	8.3kJPN chr11:111631612 rs115633648	-	0.0027	0.9987	-
148_GLY	VAL	8.3kJPN chr11:111631600 -	-	0.0001	0.9897	-
161_VAL	MET	gnomAD chr19:52714726 rs146859239	-	0.000116821	0.1277	-
163_ALA	VAL	8.3kJPN chr19:52714733 rs749322788	-	0.0001	0.1056	-
173_CYS	TRP	ClinVar chr19:52715957 rs2122334423	Likely pathogenic	-	0.999	Neurodevelopmental disorder [MONDO:MONDO:0700092,MeSH:D065886,MedGen:C1535926]
177_THR	ALA	ClinVar chr19:52715967 -	Likely pathogenic	-	0.9929	Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome [MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284]
178_PRO	LEU	VAR_074488 rs786205228	LP/P	-	0.9997	Intellectual developmental disorder, autosomal dominant 36 (MRD36) [MIM:616362]
178_PRO	LEU	ClinVar chr19:52715971 rs786205228	Pathogenic	-	0.9997	Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome\|not provided\|Malignant neoplasm of body of uterus\|Uterine carcinosarcoma\|PPP2R1A-related neurodevelopmental disorders\|Inborn genetic diseases [MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284\|MedGen:C3661900\|MedGen:C0153574\|MONDO:MONDO:0006485,MedGen:C0280630\|\|MeSH:D030342,MedGen:C0950123]
178_PRO	ARG	ClinVar chr19:52715971 rs786205228	Likely pathogenic	-	0.9999	Uterine carcinosarcoma\|Malignant neoplasm of body of uterus [MONDO:MONDO:0006485,MedGen:C0280630\|MedGen:C0153574]
178_PRO	HIS	ClinVar chr19:52715971 rs786205228	Likely pathogenic	-	1.0	Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome [MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284]
179_MET	THR	ClinVar chr19:52715974 rs1600167941	Pathogenic	-	1.0	PPP2R1A-related disorder\|Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome\|not provided [\|MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284\|MedGen:C3661900]
179_MET	ARG	ClinVar chr19:52715974 rs1600167941	Likely pathogenic	-	0.9999	See cases [-]
179_MET	VAL	ClinVar chr19:52715973 rs2122334663	Pathogenic	-	0.996	not provided\|Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome [MedGen:C3661900\|MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284]
181_ARG	TRP	VAR_074489 rs786205227	LP/P	-	0.9999	Intellectual developmental disorder, autosomal dominant 36 (MRD36) [MIM:616362]
181_ARG	TRP	ClinVar chr19:52715979 rs786205227	Pathogenic/Likely pathogenic	-	0.9999	Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome\|not provided\|Inborn genetic diseases [MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284\|MedGen:C3661900\|MeSH:D030342,MedGen:C0950123]
181_ARG	TER	8.3kJPN chr11:111630635 rs781991718	-	0.0001	-	-
182_ARG	TRP	ClinVar chr19:52715982 rs1057519946	Pathogenic/Likely pathogenic	-	0.9995	Squamous cell carcinoma of the head and neck\|Gastric adenocarcinoma\|Uterine carcinosarcoma\|Malignant neoplasm of body of uterus\|Breast neoplasm\|Ovarian serous cystadenocarcinoma\|Lung adenocarcinoma\|Neoplasm of the large intestine\|Prostate adenocarcinoma\|Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome\|not provided [MONDO:MONDO:0010150,MeSH:D000077195,MedGen:C1168401,OMIM:275355,Orphanet:67037\|Human Phenotype Ontology:HP:0033770,MONDO:MONDO:0005036,MedGen:C0278701\|MONDO:MONDO:0006485,MedGen:C0280630\|MedGen:C0153574\|Human Phenotype Ontology:HP:0010623,Human Phenotype Ontology:HP:0100013,MONDO:MONDO:0021100,MeSH:D001943,MedGen:C1458155\|MONDO:MONDO:0006046,MedGen:C0279663\|Human Phenotype Ontology:HP:0030078,MONDO:MONDO:0005061,MeSH:D000077192,MedGen:C0152013\|Human Phenotype Ontology:HP:0100834,MONDO:MONDO:0005335,MeSH:D015179,MedGen:C0009404\|MONDO:MONDO:0005082,MedGen:C0007112\|MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284\|MedGen:C3661900]
182_ARG	GLY	ClinVar chr19:52715982 rs1057519946	Likely pathogenic	-	0.9991	Prostate adenocarcinoma\|Breast neoplasm\|Squamous cell carcinoma of the head and neck\|Ovarian serous cystadenocarcinoma\|Uterine carcinosarcoma\|Malignant neoplasm of body of uterus\|Neoplasm of the large intestine\|Gastric adenocarcinoma\|Lung adenocarcinoma [MONDO:MONDO:0005082,MedGen:C0007112\|Human Phenotype Ontology:HP:0010623,Human Phenotype Ontology:HP:0100013,MONDO:MONDO:0021100,MeSH:D001943,MedGen:C1458155\|MONDO:MONDO:0010150,MeSH:D000077195,MedGen:C1168401,OMIM:275355,Orphanet:67037\|MONDO:MONDO:0006046,MedGen:C0279663\|MONDO:MONDO:0006485,MedGen:C0280630\|MedGen:C0153574\|Human Phenotype Ontology:HP:0100834,MONDO:MONDO:0005335,MeSH:D015179,MedGen:C0009404\|Human Phenotype Ontology:HP:0033770,MONDO:MONDO:0005036,MedGen:C0278701\|Human Phenotype Ontology:HP:0030078,MONDO:MONDO:0005061,MeSH:D000077192,MedGen:C0152013]
182_ARG	PRO	ClinVar chr19:52715983 rs1057519947	Likely pathogenic	-	0.9998	Inborn genetic diseases [MeSH:D030342,MedGen:C0950123]
218_SER	LEU	ClinVar chr19:52716212 rs1555791268	Pathogenic/Likely pathogenic	-	0.9996	Inborn genetic diseases\|Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome\|not provided\|PPP2R1A-related condition [MeSH:D030342,MedGen:C0950123\|MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284\|MedGen:C3661900\|]
219_VAL	MET	ClinVar chr19:52716214 rs2122337413	Pathogenic	-	0.9997	not provided [MedGen:C3661900]
219_VAL	LEU	8.3kJPN chr11:111626168 rs116572596	-	-	0.9995	-
220_ARG	CYS	gnomAD chr11:111626165 rs376765814	-	0.00011318	0.999	-
240_GLU	ASP	8.3kJPN chr11:111626103 -	-	0.0001	0.323	-
248_ARG	CYS	8.3kJPN chr19:52716301 -	-	0.0001	0.5679	-
257_ARG	HIS	VAR_074490 rs863225094	LP/P	-	0.9995	Intellectual developmental disorder, autosomal dominant 36 (MRD36) [MIM:616362]
257_ARG	HIS	ClinVar chr19:52716329 rs863225094	Pathogenic/Likely pathogenic	-	0.9995	Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome\|not provided\|9 conditions\|See cases [MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284\|MedGen:C3661900\|9 conditions\|]
257_ARG	CYS	8.3kJPN chr11:111626054 rs1283934812	-	0.0003	0.9999	-
281_ASP	GLY	8.3kJPN chr19:52719069 -	-	0.0003	0.8871	-
291_LYS	GLU	gnomAD chr11:111625752 rs562158440	-	0.000353925	0.9071	-
294_GLU	ASP	8.3kJPN chr11:111625741 rs1347203213	-	0.0017	0.9998	-
297_VAL	LEU	8.3kJPN chr19:52719116 -	-	0.0001	0.9996	-
309_GLY	ASP	gnomAD chr11:111625287 rs200276094	-	0.000112872	-	-
317_ARG	GLY	8.3kJPN chr11:111625264 rs782527776	-	0.0002	0.8395	-
318_GLU	TER	8.3kJPN chr11:111625261 -	-	0.0001	-	-
341_LYS	ARG	gnomAD chr11:111624270 rs115165302	-	0.00113841	0.2708	-
341_LYS	ARG	8.3kJPN chr11:111624270 rs115165302	-	0.0002	0.2708	-
361_ILE	THR	gnomAD chr11:111624210 rs782060653	-	0.000135349	0.3968	-
374_ASP	GLU	8.3kJPN chr19:52719913 -	-	0.0006	0.9974	-
375_GLU	ASP	8.3kJPN chr11:111624167 -	-	0.0001	0.3247	-
388_ASP	GLY	8.3kJPN chr11:111623019 -	-	0.0001	0.9504	-
398_GLN	HIS	8.3kJPN chr11:111622988 rs1386768193	-	0.0001	0.9158	-
427_PRO	LEU	8.3kJPN chr11:111622902 rs1555048136	-	0.0001	0.9991	-
456_ALA	THR	gnomAD chr11:111614249 rs144458207	-	0.000437593	0.9326	-
477_ALA	VAL	8.3kJPN chr19:52724301 -	-	0.0001	0.9761	-
490_GLY	ARG	8.3kJPN chr19:52724339 rs969720045	-	0.0002	0.2603	-
497_ARG	LEU	ClinVar chr19:52724361 -	Likely pathogenic	-	0.9995	Microcephaly-corpus callosum hypoplasia-intellectual disability-facial dysmorphism syndrome [MONDO:MONDO:0014605,MedGen:C4225352,OMIM:616362,Orphanet:457284]
500_THR	MET	8.3kJPN chr19:52724370 -	-	0.0001	0.2526	-
512_GLY	ARG	8.3kJPN chr19:52725370 rs770604606	-	0.0002	0.8908	-
523_THR	MET	8.3kJPN chr19:52725404 rs747537922	-	0.0001	0.1143	-
526_ARG	CYS	8.3kJPN chr19:52725412 rs770194852	-	0.0001	0.1308	-
528_ALA	THR	8.3kJPN chr19:52725418 -	-	0.0001	0.2595	-
536_ARG	CYS	gnomAD chr11:111613299 rs566998075	-	0.000119403	0.9744	-
557_SER	ASN	8.3kJPN chr19:52728981 rs1262874131	-	0.0001	0.0582	-
573_ASP	GLU	8.3kJPN chr11:111612808 rs143440908	-	0.0007	0.9951	-
574_VAL	ILE	8.3kJPN chr19:52729031 rs1158328586	-	0.0001	0.811	-
582_ILE	VAL	gnomAD chr11:111612783 rs115287852	-	0.00102639	-	-

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Reference

PiSite: a database of protein interaction sites using multiple binding states in the PDB, Miho Higurashi, Takashi Ishida and Kengo Kinoshita, Nucleic Acids Research 2009 37(Database issue):D360-D364

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Sequence information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Binding partner information

Variants

Reference

	P67775	Serine/threonine-protein phosphatase 2A catalytic subunit alpha isoform
	Q13362	Serine/threonine-protein phosphatase 2A 56 kDa regulatory subunit gamma isoform

	P67775	Serine/threonine-protein phosphatase 2A catalytic subunit alpha isoform, PP2A-alpha
	Q9Y5P8	Serine/threonine-protein phosphatase 2A regulatory subunit B'' subunit beta - Cell division control protein 6 homolog chimeric construct

	P67775	Serine/threonine-protein phosphatase 2A catalytic subunit alpha isoform
	P63151	Serine/threonine-protein phosphatase 2A 55 kDa regulatory subunit B alpha isoform

	P67775	Serine/threonine-protein phosphatase 2A catalytic subunit alpha isoform
	Q8BH58	TIP41-like protein

	Q13033	Striatin-3
	Q13033	Striatin-3