Protein-kinase a and human disease: The core of cAMP-dependent signaling in health and disease

C. A. Stratakis; W. R. Miller; E. Severin; K. V. Chin; J. Bertherat; P. S. Amieux; C. Eng; G. M. Kammer; J. E. Dumont; G. Tortora; M. A. Beaven; T. T. Puck; S. M. Jan de Beur; L. S. Weistein; Y. S. Cho-Chung

doi:10.1055/s-2002-26710

Protein-kinase a and human disease: The core of cAMP-dependent signaling in health and disease

C. A. Stratakis, W. R. Miller, E. Severin, K. V. Chin, J. Bertherat, P. S. Amieux, C. Eng, G. M. Kammer, J. E. Dumont, G. Tortora, M. A. Beaven, T. T. Puck, S. M. Jan de Beur, L. S. Weistein, Y. S. Cho-Chung

Research output: Contribution to journal › Review article › peer-review

3 Scopus citations

Abstract

This report summarizes several aspects of protein kinase A (PKA) signaling and its involvement in health and disease. It follows the discovery that PRKAR1A, the gene that codes for the relatively abundant regulatory subunit type 1A of protein kinase A, is the tumor suppressor gene responsible for the multiple endocrine neoplasia syndrome known as "Carney complex." Although cyclic AMP (cAMP)-dependent signaling has already been found to be involved in the pathogenesis of other genetic conditions predisposing to tumor development, this is the first time that PKA itself was directly involved in tumorigenesis and, perhaps, carcinogenesis in an inherited disease.

Original language	English (US)
Pages (from-to)	169-175
Number of pages	7
Journal	Hormone and Metabolic Research
Volume	34
Issue number	4
DOIs	https://doi.org/10.1055/s-2002-26710
State	Published - Jan 1 2002
Externally published	Yes

Keywords

Carney Complex
Chromosome 17
Endocrine Tumors
G-Proteins
Multiple Endocrine Neoplasia
Oncogene
Protein Kinase A
Regulatory Subunits
Tumor Suppressor Gene

ASJC Scopus subject areas

Endocrinology, Diabetes and Metabolism
Biochemistry
Endocrinology
Clinical Biochemistry
Biochemistry, medical

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10.1055/s-2002-26710

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Stratakis, C. A., Miller, W. R., Severin, E., Chin, K. V., Bertherat, J., Amieux, P. S., Eng, C., Kammer, G. M., Dumont, J. E., Tortora, G., Beaven, M. A., Puck, T. T., Jan de Beur, S. M., Weistein, L. S., & Cho-Chung, Y. S. (2002). Protein-kinase a and human disease: The core of cAMP-dependent signaling in health and disease. Hormone and Metabolic Research, 34(4), 169-175. https://doi.org/10.1055/s-2002-26710

Stratakis, CA, Miller, WR, Severin, E, Chin, KV, Bertherat, J, Amieux, PS, Eng, C, Kammer, GM, Dumont, JE, Tortora, G, Beaven, MA, Puck, TT, Jan de Beur, SM, Weistein, LS & Cho-Chung, YS 2002, 'Protein-kinase a and human disease: The core of cAMP-dependent signaling in health and disease', Hormone and Metabolic Research, vol. 34, no. 4, pp. 169-175. https://doi.org/10.1055/s-2002-26710

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abstract = "This report summarizes several aspects of protein kinase A (PKA) signaling and its involvement in health and disease. It follows the discovery that PRKAR1A, the gene that codes for the relatively abundant regulatory subunit type 1A of protein kinase A, is the tumor suppressor gene responsible for the multiple endocrine neoplasia syndrome known as {"}Carney complex.{"} Although cyclic AMP (cAMP)-dependent signaling has already been found to be involved in the pathogenesis of other genetic conditions predisposing to tumor development, this is the first time that PKA itself was directly involved in tumorigenesis and, perhaps, carcinogenesis in an inherited disease.",

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doi = "10.1055/s-2002-26710",

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T2 - The core of cAMP-dependent signaling in health and disease

AU - Stratakis, C. A.

AU - Miller, W. R.

AU - Severin, E.

AU - Chin, K. V.

AU - Bertherat, J.

AU - Amieux, P. S.

AU - Eng, C.

AU - Kammer, G. M.

AU - Dumont, J. E.

AU - Tortora, G.

AU - Beaven, M. A.

AU - Puck, T. T.

AU - Jan de Beur, S. M.

AU - Weistein, L. S.

AU - Cho-Chung, Y. S.

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N2 - This report summarizes several aspects of protein kinase A (PKA) signaling and its involvement in health and disease. It follows the discovery that PRKAR1A, the gene that codes for the relatively abundant regulatory subunit type 1A of protein kinase A, is the tumor suppressor gene responsible for the multiple endocrine neoplasia syndrome known as "Carney complex." Although cyclic AMP (cAMP)-dependent signaling has already been found to be involved in the pathogenesis of other genetic conditions predisposing to tumor development, this is the first time that PKA itself was directly involved in tumorigenesis and, perhaps, carcinogenesis in an inherited disease.

AB - This report summarizes several aspects of protein kinase A (PKA) signaling and its involvement in health and disease. It follows the discovery that PRKAR1A, the gene that codes for the relatively abundant regulatory subunit type 1A of protein kinase A, is the tumor suppressor gene responsible for the multiple endocrine neoplasia syndrome known as "Carney complex." Although cyclic AMP (cAMP)-dependent signaling has already been found to be involved in the pathogenesis of other genetic conditions predisposing to tumor development, this is the first time that PKA itself was directly involved in tumorigenesis and, perhaps, carcinogenesis in an inherited disease.

KW - Carney Complex

KW - Chromosome 17

KW - Endocrine Tumors

KW - G-Proteins

KW - Multiple Endocrine Neoplasia

KW - Oncogene

KW - Protein Kinase A

KW - Regulatory Subunits

KW - Tumor Suppressor Gene

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DO - 10.1055/s-2002-26710

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AN - SCOPUS:18444408096

SN - 0018-5043

VL - 34

SP - 169

EP - 175

JO - Hormone and Metabolic Research

JF - Hormone and Metabolic Research

IS - 4

ER -

Protein-kinase a and human disease: The core of cAMP-dependent signaling in health and disease

Abstract

Keywords

ASJC Scopus subject areas

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