T126B_MOUSE
ID T126B_MOUSE Reviewed; 230 AA.
AC Q9D1R1;
DT 20-MAR-2007, integrated into UniProtKB/Swiss-Prot.
DT 01-JUN-2001, sequence version 1.
DT 03-AUG-2022, entry version 117.
DE RecName: Full=Complex I assembly factor TMEM126B, mitochondrial {ECO:0000305};
DE AltName: Full=Transmembrane protein 126B;
GN Name=Tmem126b {ECO:0000312|MGI:MGI:1915722};
OS Mus musculus (Mouse).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae;
OC Murinae; Mus; Mus.
OX NCBI_TaxID=10090;
RN [1]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC STRAIN=C57BL/6J; TISSUE=Embryo;
RX PubMed=16141072; DOI=10.1126/science.1112014;
RA Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N.,
RA Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K.,
RA Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.,
RA Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R.,
RA Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T.,
RA Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A.,
RA Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B.,
RA Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M.,
RA Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S.,
RA Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E.,
RA Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D.,
RA Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M.,
RA Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H.,
RA Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V.,
RA Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S.,
RA Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H.,
RA Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N.,
RA Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F.,
RA Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G.,
RA Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z.,
RA Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C.,
RA Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y.,
RA Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S.,
RA Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K.,
RA Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R.,
RA van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H.,
RA Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M.,
RA Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C.,
RA Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S.,
RA Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K.,
RA Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M.,
RA Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C.,
RA Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A.,
RA Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.;
RT "The transcriptional landscape of the mammalian genome.";
RL Science 309:1559-1563(2005).
RN [2]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC TISSUE=Brain;
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 IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
RC TISSUE=Brown adipose tissue, Heart, Liver, and Spleen;
RX PubMed=21183079; DOI=10.1016/j.cell.2010.12.001;
RA Huttlin E.L., Jedrychowski M.P., Elias J.E., Goswami T., Rad R.,
RA Beausoleil S.A., Villen J., Haas W., Sowa M.E., Gygi S.P.;
RT "A tissue-specific atlas of mouse protein phosphorylation and expression.";
RL Cell 143:1174-1189(2010).
CC -!- FUNCTION: As part of the MCIA complex, involved in the assembly of the
CC mitochondrial complex I. Participates in constructing the membrane arm
CC of complex I. {ECO:0000250|UniProtKB:Q8IUX1}.
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. Associates with the
CC intermediate 370 kDa subcomplex of incompletely assembled complex I.
CC Interacts with TMEM70 (By similarity). {ECO:0000250|UniProtKB:Q8IUX1}.
CC -!- SUBCELLULAR LOCATION: Mitochondrion membrane {ECO:0000250}; Multi-pass
CC membrane protein {ECO:0000250}.
CC -!- SIMILARITY: Belongs to the TMEM126 family. {ECO:0000305}.
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DR EMBL; AK003201; BAB22637.1; -; mRNA.
DR EMBL; BC049680; AAH49680.1; -; mRNA.
DR CCDS; CCDS21448.1; -.
DR RefSeq; NP_081010.1; NM_026734.1.
DR AlphaFoldDB; Q9D1R1; -.
DR STRING; 10090.ENSMUSP00000032843; -.
DR PhosphoSitePlus; Q9D1R1; -.
DR EPD; Q9D1R1; -.
DR jPOST; Q9D1R1; -.
DR MaxQB; Q9D1R1; -.
DR PaxDb; Q9D1R1; -.
DR PeptideAtlas; Q9D1R1; -.
DR PRIDE; Q9D1R1; -.
DR ProteomicsDB; 253456; -.
DR Antibodypedia; 17580; 56 antibodies from 13 providers.
DR DNASU; 68472; -.
DR Ensembl; ENSMUST00000032843; ENSMUSP00000032843; ENSMUSG00000030614.
DR GeneID; 68472; -.
DR KEGG; mmu:68472; -.
DR UCSC; uc009ihp.1; mouse.
DR CTD; 55863; -.
DR MGI; MGI:1915722; Tmem126b.
DR VEuPathDB; HostDB:ENSMUSG00000030614; -.
DR eggNOG; ENOG502SQEZ; Eukaryota.
DR GeneTree; ENSGT00520000055616; -.
DR HOGENOM; CLU_105475_0_0_1; -.
DR InParanoid; Q9D1R1; -.
DR OMA; QHYARFE; -.
DR OrthoDB; 1358177at2759; -.
DR PhylomeDB; Q9D1R1; -.
DR TreeFam; TF327069; -.
DR Reactome; R-MMU-6799198; Complex I biogenesis.
DR BioGRID-ORCS; 68472; 9 hits in 72 CRISPR screens.
DR ChiTaRS; Tmem126b; mouse.
DR PRO; PR:Q9D1R1; -.
DR Proteomes; UP000000589; Chromosome 7.
DR RNAct; Q9D1R1; protein.
DR Bgee; ENSMUSG00000030614; Expressed in interventricular septum and 210 other tissues.
DR Genevisible; Q9D1R1; MM.
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; ISO:MGI.
DR GO; GO:0032981; P:mitochondrial respiratory chain complex I assembly; ISO:MGI.
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..230
FT /note="Complex I assembly factor TMEM126B, mitochondrial"
FT /id="PRO_0000280717"
FT TRANSMEM 70..92
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TRANSMEM 107..126
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TRANSMEM 139..161
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TRANSMEM 196..218
FT /note="Helical"
FT /evidence="ECO:0000255"
SQ SEQUENCE 230 AA; 25444 MW; BB60044B7A417002 CRC64;
MAASQRPSWS ESKVAGVVQE GNREAPQDIK MALYKHGQLI PSLGDAKFRS PIISEIIEKK
FEHYRNDKTL NIHGTLVFGT SSSLSGIMAN LVFRNSFKVK YEALKTYASL TTLPVLATIV
SYKLFVTDAL QSGDISKESC VLRSALIGMA CGVSYPSALA FYKNGRLAVK YQTVPLPPKG
RVMLHWLLLC QTGMKAMAIP LFFQIVMGAF TGLHHYNICE KPRARLVPDD
