T126B_RAT
ID T126B_RAT Reviewed; 229 AA.
AC B2RZD2; G3V8Z8;
DT 01-MAY-2013, integrated into UniProtKB/Swiss-Prot.
DT 01-MAY-2013, sequence version 2.
DT 03-AUG-2022, entry version 66.
DE RecName: Full=Complex I assembly factor TMEM126B, mitochondrial {ECO:0000305};
DE AltName: Full=Transmembrane protein 126B;
GN Name=Tmem126b {ECO:0000312|RGD:1308371};
OS Rattus norvegicus (Rat).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae;
OC Murinae; Rattus.
OX NCBI_TaxID=10116;
RN [1]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RC STRAIN=Brown Norway;
RX PubMed=15057822; DOI=10.1038/nature02426;
RA Gibbs R.A., Weinstock G.M., Metzker M.L., Muzny D.M., Sodergren E.J.,
RA Scherer S., Scott G., Steffen D., Worley K.C., Burch P.E., Okwuonu G.,
RA Hines S., Lewis L., Deramo C., Delgado O., Dugan-Rocha S., Miner G.,
RA Morgan M., Hawes A., Gill R., Holt R.A., Adams M.D., Amanatides P.G.,
RA Baden-Tillson H., Barnstead M., Chin S., Evans C.A., Ferriera S.,
RA Fosler C., Glodek A., Gu Z., Jennings D., Kraft C.L., Nguyen T.,
RA Pfannkoch C.M., Sitter C., Sutton G.G., Venter J.C., Woodage T., Smith D.,
RA Lee H.-M., Gustafson E., Cahill P., Kana A., Doucette-Stamm L.,
RA Weinstock K., Fechtel K., Weiss R.B., Dunn D.M., Green E.D.,
RA Blakesley R.W., Bouffard G.G., De Jong P.J., Osoegawa K., Zhu B., Marra M.,
RA Schein J., Bosdet I., Fjell C., Jones S., Krzywinski M., Mathewson C.,
RA Siddiqui A., Wye N., McPherson J., Zhao S., Fraser C.M., Shetty J.,
RA Shatsman S., Geer K., Chen Y., Abramzon S., Nierman W.C., Havlak P.H.,
RA Chen R., Durbin K.J., Egan A., Ren Y., Song X.-Z., Li B., Liu Y., Qin X.,
RA Cawley S., Cooney A.J., D'Souza L.M., Martin K., Wu J.Q.,
RA Gonzalez-Garay M.L., Jackson A.R., Kalafus K.J., McLeod M.P.,
RA Milosavljevic A., Virk D., Volkov A., Wheeler D.A., Zhang Z., Bailey J.A.,
RA Eichler E.E., Tuzun E., Birney E., Mongin E., Ureta-Vidal A., Woodwark C.,
RA Zdobnov E., Bork P., Suyama M., Torrents D., Alexandersson M., Trask B.J.,
RA Young J.M., Huang H., Wang H., Xing H., Daniels S., Gietzen D., Schmidt J.,
RA Stevens K., Vitt U., Wingrove J., Camara F., Mar Alba M., Abril J.F.,
RA Guigo R., Smit A., Dubchak I., Rubin E.M., Couronne O., Poliakov A.,
RA Huebner N., Ganten D., Goesele C., Hummel O., Kreitler T., Lee Y.-A.,
RA Monti J., Schulz H., Zimdahl H., Himmelbauer H., Lehrach H., Jacob H.J.,
RA Bromberg S., Gullings-Handley J., Jensen-Seaman M.I., Kwitek A.E.,
RA Lazar J., Pasko D., Tonellato P.J., Twigger S., Ponting C.P., Duarte J.M.,
RA Rice S., Goodstadt L., Beatson S.A., Emes R.D., Winter E.E., Webber C.,
RA Brandt P., Nyakatura G., Adetobi M., Chiaromonte F., Elnitski L.,
RA Eswara P., Hardison R.C., Hou M., Kolbe D., Makova K., Miller W.,
RA Nekrutenko A., Riemer C., Schwartz S., Taylor J., Yang S., Zhang Y.,
RA Lindpaintner K., Andrews T.D., Caccamo M., Clamp M., Clarke L., Curwen V.,
RA Durbin R.M., Eyras E., Searle S.M., Cooper G.M., Batzoglou S., Brudno M.,
RA Sidow A., Stone E.A., Payseur B.A., Bourque G., Lopez-Otin C., Puente X.S.,
RA Chakrabarti K., Chatterji S., Dewey C., Pachter L., Bray N., Yap V.B.,
RA Caspi A., Tesler G., Pevzner P.A., Haussler D., Roskin K.M., Baertsch R.,
RA Clawson H., Furey T.S., Hinrichs A.S., Karolchik D., Kent W.J.,
RA Rosenbloom K.R., Trumbower H., Weirauch M., Cooper D.N., Stenson P.D.,
RA Ma B., Brent M., Arumugam M., Shteynberg D., Copley R.R., Taylor M.S.,
RA Riethman H., Mudunuri U., Peterson J., Guyer M., Felsenfeld A., Old S.,
RA Mockrin S., Collins F.S.;
RT "Genome sequence of the Brown Norway rat yields insights into mammalian
RT evolution.";
RL Nature 428:493-521(2004).
RN [2]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC TISSUE=Pituitary;
RX PubMed=15489334; DOI=10.1101/gr.2596504;
RG The MGC Project Team;
RT "The status, quality, and expansion of the NIH full-length cDNA project:
RT the Mammalian Gene Collection (MGC).";
RL Genome Res. 14:2121-2127(2004).
RN [3]
RP DISRUPTION PHENOTYPE, FUNCTION, SUBCELLULAR LOCATION, AND SUBUNIT.
RX PubMed=22982022; DOI=10.1016/j.cmet.2012.08.009;
RA Heide H., Bleier L., Steger M., Ackermann J., Drose S., Schwamb B.,
RA Zornig M., Reichert A.S., Koch I., Wittig I., Brandt U.;
RT "Complexome profiling identifies TMEM126B as a component of the
RT mitochondrial complex I assembly complex.";
RL Cell Metab. 16:538-549(2012).
CC -!- FUNCTION: As part of the MCIA complex, involved in the assembly of the
CC mitochondrial complex I (PubMed:22982022). Participates in constructing
CC the membrane arm of complex I (By similarity).
CC {ECO:0000250|UniProtKB:Q8IUX1, ECO:0000269|PubMed:22982022}.
CC -!- SUBUNIT: Part of the mitochondrial complex I assembly/MCIA complex that
CC comprises at least the core subunits TMEM126B, NDUFAF1, ECSIT and ACAD9
CC and complement subunits such as COA1 and TMEM186 (PubMed:22982022).
CC Associates with the intermediate 370 kDa subcomplex of incompletely
CC assembled complex I (By similarity). Interacts with TMEM70 (By
CC similarity). {ECO:0000250|UniProtKB:Q8IUX1,
CC ECO:0000269|PubMed:22982022}.
CC -!- SUBCELLULAR LOCATION: Mitochondrion membrane
CC {ECO:0000269|PubMed:22982022}; Multi-pass membrane protein
CC {ECO:0000269|PubMed:22982022}.
CC -!- DISRUPTION PHENOTYPE: Reduction in respiration by about two-thirds,
CC only when the electrons were fed into complex I via the NADH-linked
CC substrates malate and glutamate in ADP-stimulated mitochondria.
CC {ECO:0000269|PubMed:22982022}.
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DR EMBL; CH473956; EDM18534.1; -; Genomic_DNA.
DR EMBL; BC167111; AAI67111.1; -; mRNA.
DR RefSeq; NP_001099750.2; NM_001106280.2.
DR AlphaFoldDB; B2RZD2; -.
DR STRING; 10116.ENSRNOP00000031739; -.
DR PaxDb; B2RZD2; -.
DR PeptideAtlas; B2RZD2; -.
DR Ensembl; ENSRNOT00000038279; ENSRNOP00000031739; ENSRNOG00000022732.
DR GeneID; 293114; -.
DR KEGG; rno:293114; -.
DR CTD; 55863; -.
DR RGD; 1308371; Tmem126b.
DR eggNOG; ENOG502SQEZ; Eukaryota.
DR GeneTree; ENSGT00520000055616; -.
DR HOGENOM; CLU_105475_0_0_1; -.
DR InParanoid; B2RZD2; -.
DR OMA; QHYARFE; -.
DR OrthoDB; 1358177at2759; -.
DR PhylomeDB; B2RZD2; -.
DR TreeFam; TF327069; -.
DR Reactome; R-RNO-6799198; Complex I biogenesis.
DR PRO; PR:B2RZD2; -.
DR Proteomes; UP000002494; Chromosome 1.
DR Proteomes; UP000234681; Chromosome 1.
DR Bgee; ENSRNOG00000022732; Expressed in quadriceps femoris and 20 other tissues.
DR Genevisible; B2RZD2; RN.
DR GO; GO:0016021; C:integral component of membrane; IEA:UniProtKB-KW.
DR GO; GO:0031966; C:mitochondrial membrane; IEA:UniProtKB-SubCell.
DR GO; GO:0005739; C:mitochondrion; IBA:GO_Central.
DR GO; GO:0032981; P:mitochondrial respiratory chain complex I assembly; ISO:RGD.
DR InterPro; IPR009801; TMEM126.
DR PANTHER; PTHR16296; PTHR16296; 1.
DR Pfam; PF07114; TMEM126; 1.
PE 1: Evidence at protein level;
KW Chaperone; Membrane; Mitochondrion; Reference proteome; Transmembrane;
KW Transmembrane helix.
FT CHAIN 1..229
FT /note="Complex I assembly factor TMEM126B, mitochondrial"
FT /id="PRO_0000422165"
FT TRANSMEM 71..91
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TRANSMEM 109..129
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TRANSMEM 140..160
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TRANSMEM 198..218
FT /note="Helical"
FT /evidence="ECO:0000255"
FT CONFLICT 44
FT /note="D -> E (in Ref. 2; AAI67111)"
FT /evidence="ECO:0000305"
SQ SEQUENCE 229 AA; 25213 MW; A25AA272F6545D83 CRC64;
MASERPTGSE SGDAGVVLEG NREASQDIKM ALYKHGRLIP SLGDAKFGRP MIAEILEKKF
ESYRNDQTLN IRGTLFFGVS SSLSGVMANL VFRYSFKVKY EALRTYASLT TLPFVATAVT
YKLFVTDALQ SGNISQESCV LRSSLIGVAC GVSYPSALAF YKNGRLAVKY HTVPVPPKGR
VMLHWLLLCQ TGMKAMAVPL LFQIIFGVFN GLYHYAVCEK AYARIVPDD
