Reviewed, UniProtKB/Swiss-Prot Q9UBK2 (PRGC1_HUMAN)

Names and origin

Protein names

Recommended name:
    Peroxisome proliferator-activated receptor gamma coactivator 1-alpha
      Short name=PPAR-gamma coactivator 1-alpha
      Short name=PPARGC-1-alpha
      Short name=PGC-1-alpha
Alternative name(s):
    Ligand effect modulator 6

Gene names

Name:	PPARGC1A
Synonyms:	LEM6, PGC1, PGC1A, PPARGC1

Organism

Homo sapiens (Human) [Complete proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

Protein attributes

Sequence length	798 AA.
Sequence status	Complete.
Protein existence	Evidence at protein level.

General annotation (Comments)

Function	Transcriptional coactivator for steroid receptors and nuclear receptors. Greatly increases the transcriptional activity of PPARG and thyroid hormone receptor on the uncoupling protein promoter. Can regulate key mitochondrial genes that contribute to the program of adaptive thermogenesis By similarity. Ref.3
Subunit structure	Binds MYBBP1A, which inhibits transcriptional activation by this protein By similarity. Interacts with PRDM16 By similarity. Interacts with LRPPRC. Homooligomer. Interacts with LPIN1 By similarity. Ref.3 Ref.4
Subcellular location	Nucleus Ref.3.
Tissue specificity	Heart, skeletal muscle, liver and kidney. Expressed at lower levels in brain and pancreas and at very low levels in the intestine and white adipose tissue. In skeletal muscle, levels were lower in obese than in lean subjects and fasting induced a 2-fold increase in levels in the skeletal muscle in obese subjects. Ref.3 Ref.1 Ref.2
Sequence similarities	Contains 1 RRM (RNA recognition motif) domain.

Ontologies

Keywords
Biological process	Transcription Transcription regulation
Cellular component	Nucleus
Coding sequence diversity	Polymorphism
Ligand	RNA-binding
Molecular function	Activator
Technical term	3D-structure Complete proteome
Gene Ontology (GO)
Biological process	RNA splicing Traceable author statement. Source: UniProtKB androgen receptor signaling pathway Non-traceable author statement. Source: UniProtKB brown fat cell differentiation Traceable author statement. Source: UniProtKB cellular glucose homeostasis Non-traceable author statement. Source: UniProtKB digestion Ref.1 Traceable author statement. Source: UniProtKB fatty acid oxidation Non-traceable author statement. Source: UniProtKB gluconeogenesis Non-traceable author statement. Source: UniProtKB mRNA processing Traceable author statement. Source: UniProtKB mitochondrion organization Non-traceable author statement. Source: UniProtKB positive regulation of fatty acid oxidation Traceable author statement. Source: UniProtKB positive regulation of gene-specific transcription Inferred from direct assay. Source: UniProtKB positive regulation of gluconeogenesis Traceable author statement. Source: UniProtKB positive regulation of histone acetylation Traceable author statement. Source: UniProtKB positive regulation of transcription factor activity Inferred from direct assay. Source: UniProtKB protein stabilization Traceable author statement. Source: UniProtKB response to starvation Non-traceable author statement. Source: UniProtKB temperature homeostasis Traceable author statement. Source: UniProtKB transcription initiation from RNA polymerase II promoter Traceable author statement. Source: UniProtKB
Cellular component	DNA-directed RNA polymerase II, core complex Traceable author statement. Source: UniProtKB
Molecular function	DNA binding Ref.3 Traceable author statement. Source: UniProtKB RNA binding Traceable author statement. Source: UniProtKB RNA polymerase II transcription mediator activity Traceable author statement. Source: UniProtKB androgen receptor binding Non-traceable author statement. Source: UniProtKB ligand-dependent nuclear receptor transcription coactivator activity Traceable author statement. Source: UniProtKB nucleotide binding Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Binary interactions

With	Entry	#Exp.	IntAct	Notes
LRPPRC	P42704	2	EBI-765486,EBI-1050853

Sequence annotation (Features)

Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Molecule processing

Chain

1 – 798

798

Peroxisome proliferator-activated receptor gamma coactivator 1-alpha

PRO_0000081732

Regions

Domain

677 – 753

77

RRM

Region

293 – 339

47

Interaction with PPARG

Motif

144 – 148

5

LXXLL motif

Compositional bias

566 – 599

34

Arg/Ser-rich

Compositional bias

621 – 633

13

Arg/Ser-rich

Natural variations

Natural variant

482

1

G → S: dbSNP rs8192678. Ref.2

VAR_018450

Natural variant

612

1

T → M: dbSNP rs3736265.

VAR_018451

Secondary structure

1

.

.

.

798

Helix Strand Turn

Details...

Helix

209 – 214

6

Sequences

	Sequence		Length	Mass (Da)
	Q9UBK2-1 [UniParc]. Last modified May 1, 2000. Version 1. Checksum: 97DDD3643051A5F1	FASTA	798	91,027
	10 20 30 40 50 60 MAWDMCNQDS ESVWSDIECA ALVGEDQPLC PDLPELDLSE LDVNDLDTDS FLGGLKWCSD 70 80 90 100 110 120 QSEIISNQYN NEPSNIFEKI DEENEANLLA VLTETLDSLP VDEDGLPSFD ALTDGDVTTD 130 140 150 160 170 180 NEASPSSMPD GTPPPQEAEE PSLLKKLLLA PANTQLSYNE CSGLSTQNHA NHNHRIRTNP 190 200 210 220 230 240 AIVKTENSWS NKAKSICQQQ KPQRRPCSEL LKYLTTNDDP PHTKPTENRN SSRDKCTSKK 250 260 270 280 290 300 KSHTQSQSQH LQAKPTTLSL PLTPESPNDP KGSPFENKTI ERTLSVELSG TAGLTPPTTP 310 320 330 340 350 360 PHKANQDNPF RASPKLKSSC KTVVPPPSKK PRYSESSGTQ GNNSTKKGPE QSELYAQLSK 370 380 390 400 410 420 SSVLTGGHEE RKTKRPSLRL FGDHDYCQSI NSKTEILINI SQELQDSRQL ENKDVSSDWQ 430 440 450 460 470 480 GQICSSTDSD QCYLRETLEA SKQVSPCSTR KQLQDQEIRA ELNKHFGHPS QAVFDDEADK 490 500 510 520 530 540 TGELRDSDFS NEQFSKLPMF INSGLAMDGL FDDSEDESDK LSYPWDGTQS YSLFNVSPSC 550 560 570 580 590 600 SSFNSPCRDS VSPPKSLFSQ RPQRMRSRSR SFSRHRSCSR SPYSRSRSRS PGSRSSSRSC 610 620 630 640 650 660 YYYESSHYRH RTHRNSPLYV RSRSRSPYSR RPRYDSYEEY QHERLKREEY RREYEKRESE 670 680 690 700 710 720 RAKQRERQRQ KAIEERRVIY VGKIRPDTTR TELRDRFEVF GEIEECTVNL RDDGDSYGFI 730 740 750 760 770 780 TYRYTCDAFA ALENGYTLRR SNETDFELYF CGRKQFFKSN YADLDSNSDD FDPASTKSKY 790 DSLDFDSLLK EAQRSLRR

References

[1]	"Human peroxisome proliferator activated receptor gamma coactivator 1 (PPARGC1) gene: cDNA sequence, genomic organization, chromosomal localization, and tissue expression." Esterbauer H., Oberkofler H., Krempler F., Patsch W. Genomics 62:98-102(1999) [PubMed: 10585775] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA], TISSUE SPECIFICITY.
[2]	"Cloning and mRNA tissue distribution of human PPARgamma coactivator-1." Larrouy D., Vidal H., Andreelli F., Laville M., Langin D. Int. J. Obes. Relat. Metab. Disord. 23:1327-1332(1999) [PubMed: 10643692] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], TISSUE SPECIFICITY, VARIANT SER-482. Tissue: Skeletal muscle.
[3]	"A tissue-specific coactivator of steroid receptors, identified in a functional genetic screen." Knutti D., Kaul A., Kralli A. Mol. Cell. Biol. 20:2411-2422(2000) [PubMed: 10713165] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], FUNCTION, SUBUNIT, SUBCELLULAR LOCATION, TISSUE SPECIFICITY.
[4]	"Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex." Cooper M.P., Qu L., Rohas L.M., Lin J., Yang W., Erdjument-Bromage H., Tempst P., Spiegelman B.M. Genes Dev. 20:2996-3009(2006) [PubMed: 17050673] [Abstract] Cited for: INTERACTION WITH LRPPRC.
+	Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ

AF108205 , AF108193, AF108194, AF108195, AF108196, AF108197, AF108198, AF108199, AF108200, AF108201, AF108202, AF108203, AF108204 Genomic DNA. Translation: AAF19083.1.
AF106698 mRNA. Translation: AAF18573.1.
AF159714 mRNA. Translation: AAD51615.1.
AF186379 mRNA. Translation: AAD56250.1.

IPI

IPI00033138.

RefSeq

NP_037393.1.

UniGene

Hs.527078

3D structure databases

PDBe
RCSB PDB
PDBj

Entry	Method	Resolution (Å)	Chain	Positions	PDBsum
1XB7	X-ray	2.50	P	205-216	[»]
3CS8	X-ray	2.30	B	141-152	[»]
3D24	X-ray	2.11	B/D	198-219	[»]

SMR

Q9UBK2. Positions 675-739.

ModBase

Search...

Protein-protein interaction databases

IntAct

Q9UBK2. 2 interactions.

STRING

Q9UBK2.

PTM databases

PhosphoSite

Q9UBK2.

Proteomic databases

PRIDE

Q9UBK2.

Genome annotation databases

Ensembl

ENST00000264867; ENSP00000264867; ENSG00000109819; Homo sapiens. [Genome view]

GeneID

10891.

KEGG

hsa:10891.

UCSC

uc003gqs.1. human.

Organism-specific databases

CTD

10891.

GeneCards

GC04M023469.

H-InvDB

HIX0004134.

HGNC

HGNC:9237. PPARGC1A.

MIM

604517. gene.

PharmGKB

PA33558.

GenAtlas

Search...

Phylogenomic databases

HOGENOM

HBG716332.

HOVERGEN

Q9UBK2.

InParanoid

Q9UBK2.

OMA

RLFGDHD.

OrthoDB

EOG9ZPHF4.

PhylomeDB

Q9UBK2.

Enzyme and pathway databases

Pathway_Interaction_DB

hdac_classiii_pathway. Signaling events mediated by HDAC Class III.
p38alphabetadownstreampathway. Signaling mediated by p38-alpha and p38-beta.

Gene expression databases

ArrayExpress

Q9UBK2.

Bgee

Q9UBK2.

CleanEx

HS_PPARGC1A.

Genevestigator

Q9UBK2.

GermOnline

ENSG00000109819. Homo sapiens.

Family and domain databases

InterPro

IPR012677. a_b_plait_nuc_bd.
IPR000504. RRM_RNP1.
[Graphical view]

Gene3D

G3DSA:3.30.70.330. a_b_plait_nuc_bd. 1 hit.

Pfam

PF00076. RRM_1. 1 hit.
[Graphical view]

SMART

SM00360. RRM. 1 hit.
[Graphical view]

PROSITE

PS50102. RRM. 1 hit.
[Graphical view]

ProtoNet

Search...

Other Resources

NextBio

41353.

SOURCE

Search...

Entry information

Entry name

PRGC1_HUMAN

Accession

Primary (citable) accession number: Q9UBK2
Secondary accession number(s): Q9UN32

Entry history

Integrated into UniProtKB/Swiss-Prot:	May 10, 2004
Last sequence update:	May 1, 2000
Last modified:	February 9, 2010
This is version 82 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)

Disclaimer

Any 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.