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Protein

Cytosolic carboxypeptidase 4

Gene

AGBL1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Metallocarboxypeptidase that mediates deglutamylation of target proteins. Catalyzes the deglutamylation of polyglutamate side chains generated by post-translational polyglutamylation in proteins such as tubulins. Also removes gene-encoded polyglutamates from the carboxy-terminus of target proteins such as MYLK. Acts as a long-chain deglutamylase and specifically shortens long polyglutamate chains, while it is not able to remove the branching point glutamate, a process catalyzed by AGBL5/CCP5.By similarity

Cofactori

Zn2+By similarityNote: Binds 1 zinc ion per subunit.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi758ZincBy similarity1
Metal bindingi761ZincBy similarity1
Active sitei808NucleophileBy similarity1
Metal bindingi855ZincBy similarity1

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Carboxypeptidase, Hydrolase, Metalloprotease, Protease

Keywords - Ligandi

Metal-binding, Zinc

Enzyme and pathway databases

BioCyciZFISH:ENSG00000166748-MONOMER.

Names & Taxonomyi

Protein namesi
Recommended name:
Cytosolic carboxypeptidase 4 (EC:3.4.17.-)
Alternative name(s):
ATP/GTP-binding protein-like 1
Gene namesi
Name:AGBL1
Synonyms:CCP4
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 15

Organism-specific databases

HGNCiHGNC:26504. AGBL1.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm

Pathology & Biotechi

Involvement in diseasei

Corneal dystrophy, Fuchs endothelial, 8 (FECD8)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA corneal disease caused by loss of endothelium of the central cornea. It is characterized by focal wart-like guttata that arise from Descemet membrane and develop in the central cornea, epithelial blisters, reduced vision and pain. Descemet membrane is thickened by abnormal collagenous deposition.
See also OMIM:615523
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_070225990C → S in FECD8; decreased TCF4-binding. 1 PublicationCorresponds to variant rs181958589dbSNPEnsembl.1
Natural variantiVAR_0702261029 – 1066Missing in FECD8; enriched in the nucleus, decreased TCF4-binding. 1 PublicationAdd BLAST38

Keywords - Diseasei

Corneal dystrophy

Organism-specific databases

DisGeNETi123624.
MalaCardsiAGBL1.
MIMi615523. phenotype.
OpenTargetsiENSG00000166748.
Orphaneti98974. Fuchs endothelial corneal dystrophy.
PharmGKBiPA134923894.

Polymorphism and mutation databases

BioMutaiAGBL1.
DMDMi158706472.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003049991 – 1066Cytosolic carboxypeptidase 4Add BLAST1066

Proteomic databases

PaxDbiQ96MI9.
PeptideAtlasiQ96MI9.
PRIDEiQ96MI9.

PTM databases

iPTMnetiQ96MI9.
PhosphoSitePlusiQ96MI9.

Expressioni

Tissue specificityi

Expressed in corneal endothelium.1 Publication

Gene expression databases

BgeeiENSG00000166748.
CleanExiHS_AGBL1.
ExpressionAtlasiQ96MI9. baseline and differential.

Organism-specific databases

HPAiCAB007760.

Interactioni

Subunit structurei

Interacts with MYLK (By similarity). Interacts with TCF4.By similarity1 Publication

GO - Molecular functioni

Protein-protein interaction databases

STRINGi9606.ENSP00000413001.

Structurei

3D structure databases

ProteinModelPortaliQ96MI9.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi251 – 284Asp-richAdd BLAST34

Sequence similaritiesi

Belongs to the peptidase M14 family.Curated

Phylogenomic databases

eggNOGiKOG3641. Eukaryota.
COG2866. LUCA.
GeneTreeiENSGT00550000074405.
HOGENOMiHOG000293310.
HOVERGENiHBG107587.
InParanoidiQ96MI9.
OMAiSCSHQLL.
OrthoDBiEOG091G01L5.
PhylomeDBiQ96MI9.
TreeFamiTF313794.

Family and domain databases

Gene3Di1.25.10.10. 1 hit.
InterProiIPR011989. ARM-like.
IPR016024. ARM-type_fold.
IPR000834. Peptidase_M14.
[Graphical view]
PfamiPF00246. Peptidase_M14. 1 hit.
[Graphical view]
SUPFAMiSSF48371. SSF48371. 2 hits.

Sequences (3)i

Sequence statusi: Complete.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q96MI9-1) [UniParc]FASTAAdd to basket

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.

« Hide

        10         20         30         40         50
MISKGGSEAL LQTLVDTART APPDYDILLP LFRLLAKVGL RDKKIGRKAL
60 70 80 90 100
ELEALDVTLI LARKNLSHGQ NLLHCLWALR VFASSVSMGA MLGINGAMEL
110 120 130 140 150
LFKVITPYTR KRTQAIRAAT EVLAALLKSK SNGRRAVNRG YVTSLLGLHQ
160 170 180 190 200
DWHSHDTANA YVQIRRGLLL CLRHIAALRS GREAFLAAQG MEILFSTTQN
210 220 230 240 250
CLDDKSMEPV ISVVLQILRQ CYPTSPLPLV TASSAYAFPV PGCITTEPPH
260 270 280 290 300
DLPEEDFEDD GDDEVDKDSD TEDGKVEDDD LETDVNKLSS KPGLDRPEEE
310 320 330 340 350
LMQYEVMCLE LSYSFEELQS KLGDDLNSEK TQYANHHHIP AAASSKQHCY
360 370 380 390 400
SKDQSSCGQE REYAVQTSLL CRVKTGRSTV HLGSKKNPGV NLYQNVQSNS
410 420 430 440 450
LRRDSSESEI PDIQASPKAD AWDVDAIFCP RMSASFSNST RTREVVKVID
460 470 480 490 500
KLLQTHLKRV PFHDPYLYMA KARRTSSVVD FKMMAFPDVW GHCPPPTTQP
510 520 530 540 550
MLERKCGVQR IRIFEDIRRL IQPSDVINKV VFSLDEPWPL QDNASNCLRF
560 570 580 590 600
FSKFESGNLR KAIQVREFEY DLLVNADVNS TQHQQWFYFK VSGMQAAIPY
610 620 630 640 650
HFNIINCEKP NSQFNYGMQP TLYSVKEALL GKPTWIRTGH EICYYKNHYR
660 670 680 690 700
QSTAVAGGAS GKCYYTLTFA VTFPHSEDVC YLAYHYPYTY TALMTHLDIL
710 720 730 740 750
EKSVNLKEVY FRQDVLCQTL GGNPCPLVTI TAMPESNSDE HLEQFRHRPY
760 770 780 790 800
QVITARVHPG ESNASWVMKG TLEFLVSSDP VARLLRENFI FKIIPMLNPD
810 820 830 840 850
GVINGNHRCS LSGEDLNRQW LSPSAHLQPT IYHAKGLLYH LSSIGRSPVV
860 870 880 890 900
FCDFHGHSQK KNVFLYGCSI KETLWQAACT VGTSTILEEV NYRTLPKILD
910 920 930 940 950
KLAPAFTMSS CSFLVEKSRA STARVVVWRE MGVSRSYTME SSYCGCNQGP
960 970 980 990 1000
YQCTQRLLER TKNERAHPVD GLQGLQFGTR ELEEMGAMFC LGLLILELKS
1010 1020 1030 1040 1050
ASCSHQLLAQ AATLLSAEED ALDQHLQRLK SSNFLPKHIW FAYHFFAITN
1060
FFKMNLLLHV SPVCDT
Note: No experimental confirmation available.
Length:1,066
Mass (Da):120,281
Last modified:October 2, 2007 - v2
Checksum:iFC08DC1C44991096
GO
Isoform 2 (identifier: Q96MI9-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-301: Missing.

Note: No experimental confirmation available.
Show »
Length:765
Mass (Da):87,153
Checksum:i9E0A227F4533D78A
GO
Isoform 3 (identifier: Q96MI9-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-269: Missing.
     270-276: DTEDGKV → MGQCYNL

Note: No experimental confirmation available.
Show »
Length:797
Mass (Da):90,789
Checksum:i640C4FC2882C7DD0
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti446V → A in BAB71299 (PubMed:14702039).Curated1
Sequence conflicti524S → G in BAB71299 (PubMed:14702039).Curated1
Sequence conflicti628A → D in BAB71299 (PubMed:14702039).Curated1
Sequence conflicti658G → R in AAI28153 (PubMed:15489334).Curated1
Sequence conflicti747H → E in BAB71299 (PubMed:14702039).Curated1
Sequence conflicti747H → E in AAI28153 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_048604417P → L.Corresponds to variant rs8029810dbSNPEnsembl.1
Natural variantiVAR_048605435S → P.Corresponds to variant rs11857527dbSNPEnsembl.1
Natural variantiVAR_070225990C → S in FECD8; decreased TCF4-binding. 1 PublicationCorresponds to variant rs181958589dbSNPEnsembl.1
Natural variantiVAR_0591951010Q → R.Corresponds to variant rs8028043dbSNPEnsembl.1
Natural variantiVAR_0702261029 – 1066Missing in FECD8; enriched in the nucleus, decreased TCF4-binding. 1 PublicationAdd BLAST38

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0281761 – 301Missing in isoform 2. 1 PublicationAdd BLAST301
Alternative sequenceiVSP_0281771 – 269Missing in isoform 3. 1 PublicationAdd BLAST269
Alternative sequenceiVSP_028178270 – 276DTEDGKV → MGQCYNL in isoform 3. 1 Publication7

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK056872 mRNA. Translation: BAB71299.1.
AC016180 Genomic DNA. No translation available.
AC107978 Genomic DNA. No translation available.
AC025842 Genomic DNA. No translation available.
AC069197 Genomic DNA. No translation available.
AC022817 Genomic DNA. No translation available.
AC012229 Genomic DNA. No translation available.
AC018950 Genomic DNA. No translation available.
AC105204 Genomic DNA. No translation available.
AC078905 Genomic DNA. No translation available.
BC128152 mRNA. Translation: AAI28153.1.
UniGeneiHs.31795.
Hs.679833.

Genome annotation databases

EnsembliENST00000635782; ENSP00000490428; ENSG00000166748. [Q96MI9-1]
KEGGihsa:123624.
UCSCiuc002blz.2. human. [Q96MI9-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK056872 mRNA. Translation: BAB71299.1.
AC016180 Genomic DNA. No translation available.
AC107978 Genomic DNA. No translation available.
AC025842 Genomic DNA. No translation available.
AC069197 Genomic DNA. No translation available.
AC022817 Genomic DNA. No translation available.
AC012229 Genomic DNA. No translation available.
AC018950 Genomic DNA. No translation available.
AC105204 Genomic DNA. No translation available.
AC078905 Genomic DNA. No translation available.
BC128152 mRNA. Translation: AAI28153.1.
UniGeneiHs.31795.
Hs.679833.

3D structure databases

ProteinModelPortaliQ96MI9.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi9606.ENSP00000413001.

PTM databases

iPTMnetiQ96MI9.
PhosphoSitePlusiQ96MI9.

Polymorphism and mutation databases

BioMutaiAGBL1.
DMDMi158706472.

Proteomic databases

PaxDbiQ96MI9.
PeptideAtlasiQ96MI9.
PRIDEiQ96MI9.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000635782; ENSP00000490428; ENSG00000166748. [Q96MI9-1]
KEGGihsa:123624.
UCSCiuc002blz.2. human. [Q96MI9-1]

Organism-specific databases

CTDi123624.
DisGeNETi123624.
GeneCardsiAGBL1.
HGNCiHGNC:26504. AGBL1.
HPAiCAB007760.
MalaCardsiAGBL1.
MIMi615496. gene.
615523. phenotype.
neXtProtiNX_Q96MI9.
OpenTargetsiENSG00000166748.
Orphaneti98974. Fuchs endothelial corneal dystrophy.
PharmGKBiPA134923894.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3641. Eukaryota.
COG2866. LUCA.
GeneTreeiENSGT00550000074405.
HOGENOMiHOG000293310.
HOVERGENiHBG107587.
InParanoidiQ96MI9.
OMAiSCSHQLL.
OrthoDBiEOG091G01L5.
PhylomeDBiQ96MI9.
TreeFamiTF313794.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000166748-MONOMER.

Miscellaneous databases

ChiTaRSiAGBL1. human.
GenomeRNAii123624.
PROiQ96MI9.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000166748.
CleanExiHS_AGBL1.
ExpressionAtlasiQ96MI9. baseline and differential.

Family and domain databases

Gene3Di1.25.10.10. 1 hit.
InterProiIPR011989. ARM-like.
IPR016024. ARM-type_fold.
IPR000834. Peptidase_M14.
[Graphical view]
PfamiPF00246. Peptidase_M14. 1 hit.
[Graphical view]
SUPFAMiSSF48371. SSF48371. 2 hits.
ProtoNetiSearch...

Entry informationi

Entry nameiCBPC4_HUMAN
AccessioniPrimary (citable) accession number: Q96MI9
Secondary accession number(s): A1A4X5, A6NJH6, C9JHL5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 2, 2007
Last sequence update: October 2, 2007
Last modified: November 2, 2016
This is version 97 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 15
    Human chromosome 15: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. Peptidase families
    Classification of peptidase families and list of entries
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.