PCK2
Ensembl | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| UniProt | |||||||||
| RefSeq (mRNA) | |||||||||
| RefSeq (protein) | |||||||||
| Location (UCSC) | Chr 14: 24.09 – 24.11 Mb | Chr 14: 55.78 – 55.79 Mb | |||||||
| PubMed search | [3] | [4] | |||||||
| View/Edit Human | View/Edit Mouse |
Phosphoenolpyruvate carboxykinase 2, mitochondrial (PCK2, PEPCK-M), is an
Alternatively spliced transcript variants have been described.[provided by RefSeq, Apr 2014][5]
Structure
The PCK2 gene encodes the mitochondrial form of PCK and shares a 68% homology in
Function
As a PCK, PCK2 catalyzes the GTP-driven conversion of OAA to PEP as a rate-limiting step in
glucose-6-phosphate (G6P) are reversible, PCK2-mediated synthesis of PEP could fuel multiple biosynthetic processes, such as serine synthesis, glycerol synthesis, and nucleotide synthesis.[10] Notably, PCK2 preferentially converts OAA derived from lactate and, thus, can promote biosynthesis even under low-glucose conditions.[6][9][10] As a result, PCK2 activity contributes to cell growth and survival during stress.[9]
While PCK1 is mainly expressed in the
leukocytes and neurons, as well as in non-gluconeogenic tissues, including pancreas, brain, heart. Moreover, while PCK1 expression is regulated by hormones or nutrients involved in gluconeogenesis, PCK2 is constitutively expressed. These differences indicate that PCK2 may also perform non-gluconeogenic functions.[6][9]
Clinical Significance
PCK2 is associated with several
cataplerotic pathways to convert TCA cycle intermediates to glycolytic intermediates, PCK2 activity can enhance the survival tumor cells facing reduced glucose levels.[9][10]
Due to the gluconeogenic function of PCK2, PCK2 deficiency is expected to disrupt glucose homeostasis and result in hypoglycemia. However, though two cases have been documented, a subsequent study suggested that PCK2 deficiency may not have been the primary cause.[7][11]
Interactive pathway map
Click on genes, proteins and metabolites below to link to respective articles.[§ 1]
Glycolysis and Gluconeogenesis edit
- ^ The interactive pathway map can be edited at WikiPathways: "GlycolysisGluconeogenesis_WP534".
See also
References
- ^ a b c ENSG00000285241 GRCh38: Ensembl release 89: ENSG00000100889, ENSG00000285241 – Ensembl, May 2017
- ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000040618 – Ensembl, May 2017
- ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- ^ "PCK2 phosphoenolpyruvate carboxykinase 2 (mitochondrial)". NCBI Entrez Gene database.
- ^ PMID 9657976.
- ^ PMID 8645161.
- PMID 15315819.
- ^ PMID 24973213.
- ^ PMID 24632615.
- PMID 19587243.