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FLASH GENE
Symbol CPT1C contributors: mct/shn - updated : 09-12-2016
HGNC name carnitine palmitoyltransferase 1C
HGNC id 18540
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N-terminal end lacks the mitochondrial import signal present in CPT1A and contains a putative microsomal targeting signal responsible for ER localization
  • two membrane-spanning region, and second transmembrane domain is longer than in the other two isoforms, which may enable it to sort proteins to the ER rather than to mitochondrial outer membrane
  • a large C terminal cytosolic domain responsible for the catalytic function
    • HOMOLOGY
    interspecies ortholog to Cpt1, Mus musculus
    ortholog to Cpt1c, Rattus Norvegicus
    intraspecies homolog to CPT1A,CPT1B
    Homologene
    FAMILY carnitine/choline acetyltransferase family
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm,organelle,mitochondria,outer
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    text
  • brain-specific isoform located in the endoplasmic reticulum of neurons
  • located in the endoplasmic reticulum throughout the neuron, even inside dendritic spines
    • basic FUNCTION
  • playing a role in neural tissue fatty acid metabolism and in feeding behavior or appetite control
  • participates in a biosynthetic pathway, facilitating the constant transport of palmitate across the ER membrane, rather than in a highly active catabolic pathway such as fatty acid oxidation
  • necessary for the regulation of energy homeostasis
  • can elicit profound effects on brain physiology and total fatty acid profiles
  • is implicated in malonyl-CoA action in leptin hypothalamic anorectic signaling pathways
  • implicated in the hypothalamic regulation of food intake and energy homeostasis
  • regulates the levels of ceramide in the endoplasmic reticulum of hippocampal neurons, and this is a relevant mechanism for the correct maturation of dendritic spines and for proper spatial learning
  • couples malonyl-CoA to ceramide levels thereby contributing to systemic energy homeostasis and feeding behavior
  • plays a role as a novel regulator of AMPAR surface expression in neurons
  • is a regulator of AMPAR translation efficiency and therefore also synaptic function in the hippocampus
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    text carnitine palmitoyltransferase activity
    PATHWAY
    metabolism lipid/lipoprotein
    signaling
    fatty and beta oxidation pathway
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein binding malonyl-CoA, and regulating malonyl-CoA availability in the brain cell
  • in addition to formerly reported mechanisms, GHRL also induces food intake through regulation of hypothalamic CPT1C and ceramide metabolism, a finding of potential importance for the understanding and treatment of obesity
  • despite its potential as a depalmitoylating enzyme, CPT1C does not affect the palmitoylation state of GRIA1
  • CPT1C binds GRIA1 and GRIA2 and that the three proteins have the same expression profile during neuronal maturation
    • cell & other
    REGULATION
    inhibited by malonyl-CoA during physiological hyperglycemia w
    ASSOCIATED DISORDERS
    corresponding disease(s) SPG73
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • CPT1c KO mice have lower body weight and food intake, exhibit decreased rates of fatty acid oxidation and are more susceptible to obesity
  • double transgenic mice CPT1c-TgN displayed severe growth retardation and a greater reduction in brain weight in the postnatal period with a stunted development at 2 weeks of age