Entry - *177040 - SERGLYCIN; SRGN - OMIM
 
* 177040

SERGLYCIN; SRGN


Alternative titles; symbols

PROTEOGLYCAN 1; PRG1; PRG
PLATELET PROTEOGLYCAN PROTEIN CORE; PPG
PROTEOGLYCAN PROTEIN CORE FOR MAST CELL SECRETORY GRANULE


HGNC Approved Gene Symbol: SRGN

Cytogenetic location: 10q22.1     Genomic coordinates (GRCh38): 10:69,087,603-69,104,811 (from NCBI)


TEXT

Description

The proteoglycans that are stored in the secretory granules of many hematopoietic cells contain a peptide core, termed serglycin (or 'serine/glycine-rich peptide core of secretory granule proteoglycan'), with a protease-resistant, glycosaminoglycan-attachment region consisting predominantly of alternating serine and glycine residues (summary by Humphries et al., 1992). Serglycin proteoglycans have been isolated that contain O-linked heparin, heparan sulfate, chondroitin sulfate A, etc.


Cloning and Expression

The promyelocytic leukemia cell line HL-60 is a transformed human cell that synthesizes chondroitin sulfate to proteoglycans and stores the proteoglycans in its secretory granules. Under certain in vitro conditions this cell can be induced to differentiate into cells that resemble neutrophils, monocyte-macrophages, eosinophils, and basophils. Stevens et al. (1988) used a rat cell-derived cDNA for a proteoglycan to isolate a cDNA from an HL-60 cell cDNA library. As assessed by Northern and Southern blot analyses, a single gene encodes the 1.3-kb mRNA for this proteoglycan peptide core. The deduced amino acid sequence includes an 18-amino acid glycosaminoglycan attachment region that consists primarily of alternating serine and glycine.

Perin et al. (1988) characterized the protein core of platelet proteoglycan and Alliel et al. (1988) determined its complete amino acid structure by a combination of protein and cDNA sequencing.

Mattei et al. (1989) found that the sequence of the peptide core of the secretory granule proteoglycan was identical to that of PPG.

Bone marrow-derived mast cells (BMMC) synthesize 200- to 250-kD highly acidic proteoglycans containing glycosaminoglycans that are almost exclusively chondroitin sulfate E. These intracellular proteoglycans are stored in secretory granules. Avraham et al. (1989) isolated and sequenced a full-length cDNA from a mouse BMMC-derived cDNA library. The same peptide core is found in all cells of bone marrow origin (Austen, 1990); see Rothenberg et al. (1988) and Avraham et al. (1988) for information on the eosinophil proteoglycan and the basophil proteoglycan, respectively. Differences in the secretory granules of the several cell types are related to the sugar moieties that are polymerized onto the glycine-serine copolymer to form the glycosoaminoglycan.

Humphries et al. (1992) determined the complete nucleotide sequence of the human serglycin gene.


Mapping

By probing DNA from a panel of human/mouse and human/hamster hybrid cells with the cDNA, Stevens et al. (1988) showed that the gene that encodes the HL-60 cell proteoglycan peptide core resides on chromosome 10. The mouse gene encoding the proteoglycan peptide core is located on chromosome 10 (Avraham et al., 1988).

Mattei et al. (1989) showed by in situ hybridization that the PRG1 gene is located in band 10q22.1.


Gene Structure

Humphries et al. (1992) determined that the human serglycin gene contains 3 exons and spans 16.7 kb. The exons and introns 1 and 2 comprise 7, 53, and 40% of the gene, respectively.


REFERENCES

  1. Alliel, P. M., Perin, J. P., Maillet, P., Bonnet, F., Rosa, J. P., Jolles, P. Complete amino acid sequence of a human platelet proteoglycan. FEBS Lett. 236: 123-126, 1988. [PubMed: 3402609, related citations] [Full Text]

  2. Austen, K. F. Personal Communication. Boston, Mass. 1/29/1990.

  3. Avraham, S., Stevens, R. L., Gartner, M. C., Austen, K. F., Lalley, P. A., Weis, J. H. Isolation of a cDNA that encodes the peptide core of the secretory granule proteoglycan of rat basophilic leukemia-1 cells and assessment of its homology to the human analogue. J. Biol. Chem. 263: 7292-7296, 1988. [PubMed: 3366780, related citations]

  4. Avraham, S., Stevens, R. L., Nicodemus, C. F., Gartner, M. C., Austen, K. F., Weis, J. H. Molecular cloning of a cDNA that encodes the peptide core of a mouse mast cell secretory granule proteoglycan and comparison with the analogous rat and human cDNA. Proc. Nat. Acad. Sci. 86: 3763-3767, 1989. [PubMed: 2726751, related citations] [Full Text]

  5. Humphries, D. E., Nicodemus, C. F., Schiller, V., Stevens, R. L. The human serglycin gene: nucleotide sequence and methylation pattern in human promyelocytic leukemia HL-60 cells and T-lymphoblast Molt-4 cells. J. Biol. Chem. 267: 13558-13563, 1992. [PubMed: 1377686, related citations]

  6. Mattei, M. G., Perin, J.-P., Alliel, P. M., Bonnet, F., Maillet, P., Passage, E., Mattei, J.-F., Jolles, P. Localization of human platelet proteoglycan gene to chromosome 10, band q22.1, by in situ hybridization. Hum. Genet. 82: 87-88, 1989. [PubMed: 2714783, related citations] [Full Text]

  7. Perin, J. P., Bonnet, F., Maillet, P., Jolles, P. Characterization and N-terminal sequence of human platelet proteoglycan. Biochem. J. 255: 1007-1013, 1988. [PubMed: 3214420, related citations] [Full Text]

  8. Rothenberg, M. E., Pomerantz, J. L., Owen, W. F., Jr., Avraham, S., Soberman, R. J., Austen, K. F., Stevens, R. L. Characterization of a human eosinophil proteoglycan, and augmentation of its biosynthesis and size by interleukin 3, interleukin 5, and granulocyte/macrophage colony stimulating factor. J. Biol. Chem. 263: 13901-13908, 1988. [PubMed: 2458354, related citations]

  9. Stevens, R. L., Avraham, S., Gartner, M. C., Bruns, G. A. P., Austen, K. F., Weis, J. H. Isolation and characterization of a cDNA that encodes the peptide core of the secretory granule proteoglycan of human promyelocytic leukemia HL-60 cells. J. Biol. Chem. 263: 7287-7291, 1988. [PubMed: 2835370, related citations]


Creation Date:
Victor A. McKusick : 3/8/1989
alopez : 12/14/2009
carol : 10/11/2001
alopez : 8/15/2000
mark : 5/14/1997
mark : 1/3/1997
mark : 7/2/1996
carol : 2/3/1995
warfield : 4/14/1994
supermim : 3/16/1992
supermim : 3/20/1990
supermim : 3/9/1990
carol : 3/7/1990

* 177040

SERGLYCIN; SRGN


Alternative titles; symbols

PROTEOGLYCAN 1; PRG1; PRG
PLATELET PROTEOGLYCAN PROTEIN CORE; PPG
PROTEOGLYCAN PROTEIN CORE FOR MAST CELL SECRETORY GRANULE


HGNC Approved Gene Symbol: SRGN

Cytogenetic location: 10q22.1     Genomic coordinates (GRCh38): 10:69,087,603-69,104,811 (from NCBI)


TEXT

Description

The proteoglycans that are stored in the secretory granules of many hematopoietic cells contain a peptide core, termed serglycin (or 'serine/glycine-rich peptide core of secretory granule proteoglycan'), with a protease-resistant, glycosaminoglycan-attachment region consisting predominantly of alternating serine and glycine residues (summary by Humphries et al., 1992). Serglycin proteoglycans have been isolated that contain O-linked heparin, heparan sulfate, chondroitin sulfate A, etc.


Cloning and Expression

The promyelocytic leukemia cell line HL-60 is a transformed human cell that synthesizes chondroitin sulfate to proteoglycans and stores the proteoglycans in its secretory granules. Under certain in vitro conditions this cell can be induced to differentiate into cells that resemble neutrophils, monocyte-macrophages, eosinophils, and basophils. Stevens et al. (1988) used a rat cell-derived cDNA for a proteoglycan to isolate a cDNA from an HL-60 cell cDNA library. As assessed by Northern and Southern blot analyses, a single gene encodes the 1.3-kb mRNA for this proteoglycan peptide core. The deduced amino acid sequence includes an 18-amino acid glycosaminoglycan attachment region that consists primarily of alternating serine and glycine.

Perin et al. (1988) characterized the protein core of platelet proteoglycan and Alliel et al. (1988) determined its complete amino acid structure by a combination of protein and cDNA sequencing.

Mattei et al. (1989) found that the sequence of the peptide core of the secretory granule proteoglycan was identical to that of PPG.

Bone marrow-derived mast cells (BMMC) synthesize 200- to 250-kD highly acidic proteoglycans containing glycosaminoglycans that are almost exclusively chondroitin sulfate E. These intracellular proteoglycans are stored in secretory granules. Avraham et al. (1989) isolated and sequenced a full-length cDNA from a mouse BMMC-derived cDNA library. The same peptide core is found in all cells of bone marrow origin (Austen, 1990); see Rothenberg et al. (1988) and Avraham et al. (1988) for information on the eosinophil proteoglycan and the basophil proteoglycan, respectively. Differences in the secretory granules of the several cell types are related to the sugar moieties that are polymerized onto the glycine-serine copolymer to form the glycosoaminoglycan.

Humphries et al. (1992) determined the complete nucleotide sequence of the human serglycin gene.


Mapping

By probing DNA from a panel of human/mouse and human/hamster hybrid cells with the cDNA, Stevens et al. (1988) showed that the gene that encodes the HL-60 cell proteoglycan peptide core resides on chromosome 10. The mouse gene encoding the proteoglycan peptide core is located on chromosome 10 (Avraham et al., 1988).

Mattei et al. (1989) showed by in situ hybridization that the PRG1 gene is located in band 10q22.1.


Gene Structure

Humphries et al. (1992) determined that the human serglycin gene contains 3 exons and spans 16.7 kb. The exons and introns 1 and 2 comprise 7, 53, and 40% of the gene, respectively.


REFERENCES

  1. Alliel, P. M., Perin, J. P., Maillet, P., Bonnet, F., Rosa, J. P., Jolles, P. Complete amino acid sequence of a human platelet proteoglycan. FEBS Lett. 236: 123-126, 1988. [PubMed: 3402609] [Full Text: https://doi.org/10.1016/0014-5793(88)80298-9]

  2. Austen, K. F. Personal Communication. Boston, Mass. 1/29/1990.

  3. Avraham, S., Stevens, R. L., Gartner, M. C., Austen, K. F., Lalley, P. A., Weis, J. H. Isolation of a cDNA that encodes the peptide core of the secretory granule proteoglycan of rat basophilic leukemia-1 cells and assessment of its homology to the human analogue. J. Biol. Chem. 263: 7292-7296, 1988. [PubMed: 3366780]

  4. Avraham, S., Stevens, R. L., Nicodemus, C. F., Gartner, M. C., Austen, K. F., Weis, J. H. Molecular cloning of a cDNA that encodes the peptide core of a mouse mast cell secretory granule proteoglycan and comparison with the analogous rat and human cDNA. Proc. Nat. Acad. Sci. 86: 3763-3767, 1989. [PubMed: 2726751] [Full Text: https://doi.org/10.1073/pnas.86.10.3763]

  5. Humphries, D. E., Nicodemus, C. F., Schiller, V., Stevens, R. L. The human serglycin gene: nucleotide sequence and methylation pattern in human promyelocytic leukemia HL-60 cells and T-lymphoblast Molt-4 cells. J. Biol. Chem. 267: 13558-13563, 1992. [PubMed: 1377686]

  6. Mattei, M. G., Perin, J.-P., Alliel, P. M., Bonnet, F., Maillet, P., Passage, E., Mattei, J.-F., Jolles, P. Localization of human platelet proteoglycan gene to chromosome 10, band q22.1, by in situ hybridization. Hum. Genet. 82: 87-88, 1989. [PubMed: 2714783] [Full Text: https://doi.org/10.1007/BF00288281]

  7. Perin, J. P., Bonnet, F., Maillet, P., Jolles, P. Characterization and N-terminal sequence of human platelet proteoglycan. Biochem. J. 255: 1007-1013, 1988. [PubMed: 3214420] [Full Text: https://doi.org/10.1042/bj2551007]

  8. Rothenberg, M. E., Pomerantz, J. L., Owen, W. F., Jr., Avraham, S., Soberman, R. J., Austen, K. F., Stevens, R. L. Characterization of a human eosinophil proteoglycan, and augmentation of its biosynthesis and size by interleukin 3, interleukin 5, and granulocyte/macrophage colony stimulating factor. J. Biol. Chem. 263: 13901-13908, 1988. [PubMed: 2458354]

  9. Stevens, R. L., Avraham, S., Gartner, M. C., Bruns, G. A. P., Austen, K. F., Weis, J. H. Isolation and characterization of a cDNA that encodes the peptide core of the secretory granule proteoglycan of human promyelocytic leukemia HL-60 cells. J. Biol. Chem. 263: 7287-7291, 1988. [PubMed: 2835370]


Creation Date:
Victor A. McKusick : 3/8/1989

Edit History:
alopez : 12/14/2009
carol : 10/11/2001
alopez : 8/15/2000
mark : 5/14/1997
mark : 1/3/1997
mark : 7/2/1996
carol : 2/3/1995
warfield : 4/14/1994
supermim : 3/16/1992
supermim : 3/20/1990
supermim : 3/9/1990
carol : 3/7/1990