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Protein

MICOS complex subunit MIC26

Gene

APOO

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

Functioni

Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. Plays a crucial role in crista junction formation and mitochondrial function (PubMed:25764979). Can promote cardiac lipotoxicity by enhancing mitochondrial respiration and fatty acid metabolism in cardiac myoblasts (PubMed:24743151). Promotes cholesterol efflux from macrophage cells. Detected in HDL, LDL and VLDL. Secreted by a microsomal triglyceride transfer protein (MTTP)-dependent mechanism, probably as a VLDL-associated protein that is subsequently transferred to HDL (PubMed:16956892).3 Publications

GO - Biological processi

  • cristae formation Source: UniProtKB
  • lipid transport Source: UniProtKB-KW
Complete GO annotation...

Keywords - Biological processi

Lipid transport, Transport

Enzyme and pathway databases

BioCyciZFISH:G66-33070-MONOMER.

Names & Taxonomyi

Protein namesi
Recommended name:
MICOS complex subunit MIC261 Publication
Alternative name(s):
Apolipoprotein O
MICOS complex subunit MIC231 Publication
Protein FAM121B
Gene namesi
Name:APOO
Synonyms:FAM121B, MIC231 Publication, MIC261 Publication
ORF Names:My025, UNQ1866/PRO4302
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome X

Organism-specific databases

HGNCiHGNC:28727. APOO.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei108 – 128HelicalSequence analysisAdd BLAST21

GO - Cellular componenti

  • endoplasmic reticulum membrane Source: UniProtKB
  • extracellular region Source: UniProtKB
  • extracellular space Source: BHF-UCL
  • Golgi membrane Source: UniProtKB
  • high-density lipoprotein particle Source: UniProtKB-KW
  • integral component of mitochondrial inner membrane Source: UniProtKB
  • low-density lipoprotein particle Source: UniProtKB-KW
  • MICOS complex Source: UniProtKB
  • mitochondrion Source: UniProtKB
  • very-low-density lipoprotein particle Source: UniProtKB-KW
Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum, Golgi apparatus, HDL, LDL, Membrane, Mitochondrion, Mitochondrion inner membrane, Secreted, VLDL

Pathology & Biotechi

Organism-specific databases

DisGeNETi79135.
OpenTargetsiENSG00000184831.
PharmGKBiPA162376709.

Polymorphism and mutation databases

BioMutaiAPOO.
DMDMi74733244.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 25Sequence analysisAdd BLAST25
ChainiPRO_000025464626 – 198MICOS complex subunit MIC26Add BLAST173

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi162O-linked (Xyl...) (chondroitin sulfate)Sequence analysis1

Post-translational modificationi

O-glycosylation; glycosaminoglycan of chondroitin-sulfate type.2 Publications

Keywords - PTMi

Glycoprotein, Proteoglycan

Proteomic databases

EPDiQ9BUR5.
MaxQBiQ9BUR5.
PaxDbiQ9BUR5.
PeptideAtlasiQ9BUR5.
PRIDEiQ9BUR5.
TopDownProteomicsiQ9BUR5-1. [Q9BUR5-1]
Q9BUR5-2. [Q9BUR5-2]

PTM databases

iPTMnetiQ9BUR5.
PhosphoSitePlusiQ9BUR5.

Expressioni

Tissue specificityi

Expressed in all tissues examined. Up-regulated in diabetic heart.1 Publication

Gene expression databases

BgeeiENSG00000184831.
CleanExiHS_APOO.
ExpressionAtlasiQ9BUR5. baseline and differential.
GenevisibleiQ9BUR5. HS.

Organism-specific databases

HPAiHPA003187.

Interactioni

Subunit structurei

Component of the mitochondrial contact site and cristae organizing system (MICOS) complex, composed of at least MINOS1/MIC10, CHCHD3/MIC19, CHCHD6/MIC25, APOOL/MIC27, IMMT/MIC60, APOO/MIC23/MIC26 and QIL1/MIC13. This complex was also known under the names MINOS or MitOS complex. The MICOS complex associates with mitochondrial outer membrane proteins SAMM50, MTX1, MTX2 and DNAJC11, mitochondrial inner membrane protein TMEM11 and with HSPA9 (PubMed:25764979, PubMed:25781180, PubMed:25997101). Interacts with IMMT/MIC60 (PubMed:25764979, PubMed:25781180). Interacts with MINOS1/MIC10 and APOOL/MIC27 (PubMed:25764979).3 Publications

Protein-protein interaction databases

BioGridi122556. 5 interactors.
IntActiQ9BUR5. 1 interactor.
STRINGi9606.ENSP00000368528.

Structurei

3D structure databases

ProteinModelPortaliQ9BUR5.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Signal, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG4798. Eukaryota.
ENOG4112AZQ. LUCA.
GeneTreeiENSGT00530000063666.
HOGENOMiHOG000034011.
HOVERGENiHBG059567.
InParanoidiQ9BUR5.
OMAiCEPYTNW.
OrthoDBiEOG091G0MR4.
PhylomeDBiQ9BUR5.
TreeFamiTF315313.

Family and domain databases

InterProiIPR019166. MIC26/MIC27.
IPR033182. MIC26/MIC27_animal.
[Graphical view]
PANTHERiPTHR14564. PTHR14564. 1 hit.
PfamiPF09769. ApoO. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q9BUR5-1) [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
MFKVIQRSVG PASLSLLTFK VYAAPKKDSP PKNSVKVDEL SLYSVPEGQS
60 70 80 90 100
KYVEEARSQL EESISQLRHY CEPYTTWCQE TYSQTKPKMQ SLVQWGLDSY
110 120 130 140 150
DYLQNAPPGF FPRLGVIGFA GLIGLLLARG SKIKKLVYPP GFMGLAASLY
160 170 180 190
YPQQAIVFAQ VSGERLYDWG LRGYIVIEDL WKENFQKPGN VKNSPGTK
Length:198
Mass (Da):22,285
Last modified:June 1, 2001 - v1
Checksum:iBEC99607F54E98E2
GO
Isoform 2 (identifier: Q9BUR5-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     80-109: ETYSQTKPKMQSLVQWGLDSYDYLQNAPPG → TAMTISKMHLLD

Show »
Length:180
Mass (Da):20,199
Checksum:i0612F903AB6390E1
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_02333380 – 109ETYSQ…NAPPG → TAMTISKMHLLD in isoform 2. 1 PublicationAdd BLAST30

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF061264 mRNA. Translation: AAG43139.1.
AY359114 mRNA. Translation: AAQ89472.1.
AK311865 mRNA. Translation: BAG34806.1.
CH471074 Genomic DNA. Translation: EAW99002.1.
BC002333 mRNA. Translation: AAH02333.1.
BC010102 mRNA. Translation: AAH10102.1.
BC016814 mRNA. Translation: AAH16814.1.
CCDSiCCDS14208.1. [Q9BUR5-1]
RefSeqiNP_077027.1. NM_024122.4. [Q9BUR5-1]
XP_016885326.1. XM_017029837.1. [Q9BUR5-1]
UniGeneiHs.495851.

Genome annotation databases

EnsembliENST00000379226; ENSP00000368528; ENSG00000184831. [Q9BUR5-1]
GeneIDi79135.
KEGGihsa:79135.
UCSCiuc004dax.4. human. [Q9BUR5-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF061264 mRNA. Translation: AAG43139.1.
AY359114 mRNA. Translation: AAQ89472.1.
AK311865 mRNA. Translation: BAG34806.1.
CH471074 Genomic DNA. Translation: EAW99002.1.
BC002333 mRNA. Translation: AAH02333.1.
BC010102 mRNA. Translation: AAH10102.1.
BC016814 mRNA. Translation: AAH16814.1.
CCDSiCCDS14208.1. [Q9BUR5-1]
RefSeqiNP_077027.1. NM_024122.4. [Q9BUR5-1]
XP_016885326.1. XM_017029837.1. [Q9BUR5-1]
UniGeneiHs.495851.

3D structure databases

ProteinModelPortaliQ9BUR5.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi122556. 5 interactors.
IntActiQ9BUR5. 1 interactor.
STRINGi9606.ENSP00000368528.

PTM databases

iPTMnetiQ9BUR5.
PhosphoSitePlusiQ9BUR5.

Polymorphism and mutation databases

BioMutaiAPOO.
DMDMi74733244.

Proteomic databases

EPDiQ9BUR5.
MaxQBiQ9BUR5.
PaxDbiQ9BUR5.
PeptideAtlasiQ9BUR5.
PRIDEiQ9BUR5.
TopDownProteomicsiQ9BUR5-1. [Q9BUR5-1]
Q9BUR5-2. [Q9BUR5-2]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000379226; ENSP00000368528; ENSG00000184831. [Q9BUR5-1]
GeneIDi79135.
KEGGihsa:79135.
UCSCiuc004dax.4. human. [Q9BUR5-1]

Organism-specific databases

CTDi79135.
DisGeNETi79135.
GeneCardsiAPOO.
HGNCiHGNC:28727. APOO.
HPAiHPA003187.
MIMi300753. gene.
neXtProtiNX_Q9BUR5.
OpenTargetsiENSG00000184831.
PharmGKBiPA162376709.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4798. Eukaryota.
ENOG4112AZQ. LUCA.
GeneTreeiENSGT00530000063666.
HOGENOMiHOG000034011.
HOVERGENiHBG059567.
InParanoidiQ9BUR5.
OMAiCEPYTNW.
OrthoDBiEOG091G0MR4.
PhylomeDBiQ9BUR5.
TreeFamiTF315313.

Enzyme and pathway databases

BioCyciZFISH:G66-33070-MONOMER.

Miscellaneous databases

ChiTaRSiAPOO. human.
GeneWikiiApolipoprotein_O.
GenomeRNAii79135.
PROiQ9BUR5.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000184831.
CleanExiHS_APOO.
ExpressionAtlasiQ9BUR5. baseline and differential.
GenevisibleiQ9BUR5. HS.

Family and domain databases

InterProiIPR019166. MIC26/MIC27.
IPR033182. MIC26/MIC27_animal.
[Graphical view]
PANTHERiPTHR14564. PTHR14564. 1 hit.
PfamiPF09769. ApoO. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiMIC26_HUMAN
AccessioniPrimary (citable) accession number: Q9BUR5
Secondary accession number(s): B2R4K9, Q9H3J9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 31, 2006
Last sequence update: June 1, 2001
Last modified: November 2, 2016
This is version 106 of the entry and version 1 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 X
    Human chromosome X: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. 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.