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FLASH GENE

Symbol

PPARGC1B

contributors: mct - updated : 21-07-2012

HGNC name	peroxisome proliferative activated receptor, gamma, coactivator 1, beta
HGNC id	30022

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

Location	5q33.1      Physical location : 149.109.814 - 149.234.584
Synonym name	PGC-1-related estrogen receptor alpha coactivator
Synonym symbol(s)	PGC1, PGC-1, PGC1B, PERC, ERRL1, PGC-1(beta)

DNA

TYPE	functioning gene
STRUCTURE	124.77 kb     13 Exon(s)

MAPPING

cloned

Y

linked

N

status

provisional

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_133263 12 - 10642 NP_573570 - 1023 - 2003 12678921 PGC1beta 1A localized to the cell nucleus PGC1beta 1B - - 3753 - 1017 - 2003 12678921 PGC1beta 2A - - 3452 - 1002 - 2003 12678921 localized to the cell nucleus NM_001172698 11 - 10525 NP_001166169 - 984 - 2003 12678921 PGC1beta 2B NM_001172699 11 - 10556 NP_001166170 - 959 - 2003 12678921

EXPRESSION

Type

widely

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol Digestive stomach         Lymphoid/Immune spleen       highly Respiratory respiratory tract larynx       Urinary bladder

tissue

System Tissue Tissue level 1 Tissue level 2 Level Pubmed Species Stage Rna symbol Muscular striatum skeletal

cell lineage

cell lines

fluid/secretion

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	RNA recognition motif
	conserved amino-acid motif that serves as a docking site for host cell factor

HOMOLOGY

intraspecies

homolog to PPARGC1

Homologene

FAMILY

CATEGORY

regulatory

SUBCELLULAR LOCALIZATION	intracellular
	intracellular,cytoplasm
	intracellular,nucleus

basic FUNCTION	involved in the regulation of hepatic gluconeogenesis and/or fatty acid oxidation during fasting
	may be playing a role in constitutive adrenergic-independent mitochondrial biogenesis
	role in mitochondrial physiology, namely the control of mitochondrial fusion mainly through MFN2
	mitochondrial biogenesis orchestrated by PPRGC1B, coupled with iron uptake through TFRC and iron supply to mitochondrial respiratory proteins, is a fundamental pathway linked to osteoclast activation and bone metabolism
	PPARGC1A and PPARGC1B control mitochondrial capacity in an additive and independent manner
	capacity of PPARGC1A and PPARGC1B to inhibit FOXO3 and NFkappaB actions and proteolysis, which helps explain how exercise prevents muscle atrophy
	regulator of the beta-oxidation of fatty acids and the oxidative phosphorylation in mitochondria
	important regulator of the APOC3 gene cluster and reveal a mechanism through which nicotinic acid achieves its therapeutic effects
	potent regulator of angiogenesis
	role of PPARGC1B is distinct from that of PPARGC1A in white adipose tissue
	PPARGC1A, and PPARGC1B modulate mitochondrial biogenesis and energy homeostasis
	plays an important role in maintaining baseline mitochondrial function and cardiac contractile function following pressure overload hypertrophy by preserving glucose metabolism and preventing oxidative stress
	plays an important role in maintaining baseline mitochondrial function and cardiac contractile function following pressure overload hypertrophy by preserving glucose metabolism and preventing oxidative stress (PMID;

CELLULAR PROCESS	cell cycle, checkpoint

PHYSIOLOGICAL PROCESS

text	cell-cycle regulation

PATHWAY

metabolism

carbohydrate , lipid/lipoprotein

signaling

a component

INTERACTION

DNA

RNA

small molecule

protein	HNF4A, PPARA (activator of HNF4A and PPARA)
	direct interaction of NRF1 and ESRRA with PPARGC1B, and their participation in mitochondrial biogenesis and respiration
	regulates plasma triglyceride metabolism through stimulating apolipoprotein C3 (APOC3) expression and elevating APOC3 levels in circulation
	CHUK and alternative NFKB regulate PPARGC1B to promote oxidative muscle metabolism
	NFE2L1 binds to the antioxidant response elements (AREs) in regulatory regions of the LPIN1 and PPARGC1B genes
	PPARGC1A, PPARGC1B coactivators regulate MITF and the tanning response
	PPARGC1B-mediated coactivation of MLXIPL is likely involved in the lipogenic response to hyperglycemia

cell & other

REGULATION

inhibited by

NFE2L1 deficiency leading to the reduced expression of the transcriptional coactivator genes LPIN1 and PPARGC1B

ASSOCIATED DISORDERS

corresponding disease(s)

Other morbid association(s)

Type Gene Modification Chromosome rearrangement Protein expression Protein Function constitutional   deletion     induces cardiac arrhythmia

Susceptibility

to obesity

Variant & Polymorphism SNP	Ala203 allele being a risk factor for obesity

Candidate gene
Marker
Therapy target
	System Type Disorder Pubmed neurology neurodegenerative   therapeutic agents activating PPARGC1A, PPARGC1B would be valuable for treating neurodegenerative diseases in which mitochondrial dysfunction and oxidative damage play an important pathogenic role

ANIMAL & CELL MODELS

Pgc-1beta deficient mice exhibited greater degrees of oxidative stress, decreased cardiac efficiency, lower rates of glucose metabolism, and repression of hexokinase II protein, when subjected to transverse aortic constriction