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Protein

MICOS complex subunit MIC26

Gene

APOO

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

Keywordsi

Biological processLipid transport, Transport

Enzyme and pathway databases

ReactomeiR-HSA-8949613 Cristae formation

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

EuPathDBiHostDB:ENSG00000184831.13
HGNCiHGNC:28727 APOO
MIMi300753 gene
neXtProtiNX_Q9BUR5

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

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

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) serineSequence 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

CarbonylDBiQ9BUR5
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. he MICOS complex associates with mitochondrial outer membrane proteins SAMM50, MTX1 and MTX2 (together described as components of the mitochondrial outer membrane sorting assembly machinery (SAM) complex) and DNAJC11, mitochondrial inner membrane protein TMEM11 and with HSPA9 (PubMed:25764979, PubMed:25781180, PubMed:25997101). The MICOS and SAM complexes together with DNAJC11 are part of a large protein complex spanning both membranes termed the mitochondrial intermembrane space bridging (MIB) complex Interacts with IMMT/MIC60 (PubMed:25764979, PubMed:25781180). Interacts with MINOS1/MIC10 and APOOL/MIC27 (PubMed:25764979).Curated3 Publications

Protein-protein interaction databases

BioGridi1225565 interactors.
CORUMiQ9BUR5
IntActiQ9BUR5 1 interactor.
MINTiQ9BUR5
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

InterProiView protein in InterPro
IPR019166 MIC26/MIC27
IPR033182 MIC26/MIC27_animal
PANTHERiPTHR14564 PTHR14564, 1 hit
PfamiView protein in Pfam
PF09769 ApoO, 1 hit

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

Similar proteinsi

Entry informationi

Entry nameiMIC26_HUMAN
AccessioniPrimary (citable) accession number: Q9BUR5
Secondary accession number(s): B2R4K9, Q9H3J9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 31, 2006
Last sequence update: June 1, 2001
Last modified: February 28, 2018
This is version 113 of the entry and version 1 of the sequence. See 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