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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
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
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
12 - 10642 - 1023 - 2003 12678921
  • PGC1beta 1A
  • localized to the cell nucleus
  • - - 3753 - 1017 - 2003 12678921
    - - 3452 - 1002 - 2003 12678921
    localized to the cell nucleus
    11 - 10525 - 984 - 2003 12678921
  • PGC1beta 2B
  • 11 - 10556 - 959 - 2003 12678921
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestivestomach    
    Lymphoid/Immunespleen   highly
    Respiratoryrespiratory tractlarynx   
    Urinarybladder    
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Muscularstriatumskeletal  
    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)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein 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
    SystemTypeDisorderPubmed
    neurologyneurodegenerative 
    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