Entry - *120105 - COLIPASE, PANCREATIC; CLPS - OMIM
 
* 120105

COLIPASE, PANCREATIC; CLPS


HGNC Approved Gene Symbol: CLPS

Cytogenetic location: 6p21.31     Genomic coordinates (GRCh38): 6:35,794,982-35,797,323 (from NCBI)


TEXT

Description

Pancreatic colipase is a 12-kD polypeptide cofactor for pancreatic lipase (EC 3.1.1.3; 246600), an enzyme essential for the absorption of dietary long-chain triglyceride fatty acids. Colipase is thought to anchor lipase noncovalently to the surface of lipid micelles, counteracting the destabilizing influence of intestinal bile salts (summary by Davis et al., 1991).


Cloning and Expression

Using primers derived from the known amino acid sequence, Davis et al. (1991) employed the PCR to produce a cDNA clone corresponding to the complete coding region of the human procolipase mRNA.


Gene Structure

Sims and Lowe (1992) found that the CLPS gene has 3 exons and that the 5-prime-flanking region contains a TATA box, a GC box, and a 28-bp region with homology to the rat pancreatic-specific enhancer.


Mapping

By the study of mouse/human somatic cell hybrids, Davis et al. (1991) determined that the CLPS gene is located in the region 6pter-p21.1.

Sims and Lowe (1992) confirmed the assignment of the CLPS gene to chromosome 6 by blots of hamster-human somatic cell hybrid DNA using the entire colipase cDNA.


Animal Model

Zhang et al. (2006) reported a mechanism by which constant darkness regulates the gene expression of fat catabolic enzymes in mice. Genes for murine procolipase (Clps) and pancreatic lipase-related protein-2 (Plrp2; 604423) are activated in a circadian manner in peripheral organs during 12-hour dark:12-hour dark (DD) but not light-dark (LD) cycles. This mechanism is deregulated in circadian-deficient Per1/Per2 double mutant mice. Zhang et al. (2006) identified circadian-regulated 5-prime-AMP, which is elevated in the blood of DD mice, as a key mediator of this response. Synthetic 5-prime-AMP induced torpor (a hibernation-like state) and murine Clps expression in LD mice. Torpor induced by metabolic stress was associated with elevated 5-prime-AMP levels in DD mice. Levels of glucose and nonesterified fatty acid in the blood were reversed in DD and LD mice. Induction of murine Clps expression by 5-prime-AMP in LD mice was reciprocally linked to blood glucose levels. Zhang et al. (2006) concluded that their findings uncovered a circadian metabolic rhythm in mammals.


REFERENCES

  1. Davis, R. C., Xia, Y., Mohandas, T., Schotz, M. C., Lusis, A. J. Assignment of the human pancreatic colipase gene to chromosome 6p21.1 to pter. Genomics 10: 262-265, 1991. [PubMed: 2045105, related citations] [Full Text]

  2. Sims, H. F., Lowe, M. E. The human colipase gene: isolation, chromosomal location, and tissue-specific expression. Biochemistry 31: 7120-7125, 1992. [PubMed: 1643046, related citations] [Full Text]

  3. Zhang, J., Kaasik, K., Blackburn, M. R., Lee, C. C. Constant darkness is a circadian metabolic signal in mammals. Nature 439: 340-343, 2006. [PubMed: 16421573, related citations] [Full Text]


Contributors:
Ada Hamosh - updated : 5/26/2006
Creation Date:
Victor A. McKusick : 2/5/1991
carol : 11/15/2011
alopez : 6/7/2006
alopez : 6/7/2006
terry : 5/26/2006
mark : 11/27/1996
carol : 10/26/1993
carol : 10/1/1992
supermim : 3/16/1992
carol : 8/7/1991
carol : 4/18/1991
carol : 2/7/1991

* 120105

COLIPASE, PANCREATIC; CLPS


HGNC Approved Gene Symbol: CLPS

Cytogenetic location: 6p21.31     Genomic coordinates (GRCh38): 6:35,794,982-35,797,323 (from NCBI)


TEXT

Description

Pancreatic colipase is a 12-kD polypeptide cofactor for pancreatic lipase (EC 3.1.1.3; 246600), an enzyme essential for the absorption of dietary long-chain triglyceride fatty acids. Colipase is thought to anchor lipase noncovalently to the surface of lipid micelles, counteracting the destabilizing influence of intestinal bile salts (summary by Davis et al., 1991).


Cloning and Expression

Using primers derived from the known amino acid sequence, Davis et al. (1991) employed the PCR to produce a cDNA clone corresponding to the complete coding region of the human procolipase mRNA.


Gene Structure

Sims and Lowe (1992) found that the CLPS gene has 3 exons and that the 5-prime-flanking region contains a TATA box, a GC box, and a 28-bp region with homology to the rat pancreatic-specific enhancer.


Mapping

By the study of mouse/human somatic cell hybrids, Davis et al. (1991) determined that the CLPS gene is located in the region 6pter-p21.1.

Sims and Lowe (1992) confirmed the assignment of the CLPS gene to chromosome 6 by blots of hamster-human somatic cell hybrid DNA using the entire colipase cDNA.


Animal Model

Zhang et al. (2006) reported a mechanism by which constant darkness regulates the gene expression of fat catabolic enzymes in mice. Genes for murine procolipase (Clps) and pancreatic lipase-related protein-2 (Plrp2; 604423) are activated in a circadian manner in peripheral organs during 12-hour dark:12-hour dark (DD) but not light-dark (LD) cycles. This mechanism is deregulated in circadian-deficient Per1/Per2 double mutant mice. Zhang et al. (2006) identified circadian-regulated 5-prime-AMP, which is elevated in the blood of DD mice, as a key mediator of this response. Synthetic 5-prime-AMP induced torpor (a hibernation-like state) and murine Clps expression in LD mice. Torpor induced by metabolic stress was associated with elevated 5-prime-AMP levels in DD mice. Levels of glucose and nonesterified fatty acid in the blood were reversed in DD and LD mice. Induction of murine Clps expression by 5-prime-AMP in LD mice was reciprocally linked to blood glucose levels. Zhang et al. (2006) concluded that their findings uncovered a circadian metabolic rhythm in mammals.


REFERENCES

  1. Davis, R. C., Xia, Y., Mohandas, T., Schotz, M. C., Lusis, A. J. Assignment of the human pancreatic colipase gene to chromosome 6p21.1 to pter. Genomics 10: 262-265, 1991. [PubMed: 2045105] [Full Text: https://doi.org/10.1016/0888-7543(91)90509-d]

  2. Sims, H. F., Lowe, M. E. The human colipase gene: isolation, chromosomal location, and tissue-specific expression. Biochemistry 31: 7120-7125, 1992. [PubMed: 1643046] [Full Text: https://doi.org/10.1021/bi00146a013]

  3. Zhang, J., Kaasik, K., Blackburn, M. R., Lee, C. C. Constant darkness is a circadian metabolic signal in mammals. Nature 439: 340-343, 2006. [PubMed: 16421573] [Full Text: https://doi.org/10.1038/nature04368]


Contributors:
Ada Hamosh - updated : 5/26/2006

Creation Date:
Victor A. McKusick : 2/5/1991

Edit History:
carol : 11/15/2011
alopez : 6/7/2006
alopez : 6/7/2006
terry : 5/26/2006
mark : 11/27/1996
carol : 10/26/1993
carol : 10/1/1992
supermim : 3/16/1992
carol : 8/7/1991
carol : 4/18/1991
carol : 2/7/1991