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KEGG NETWORK: N00327
NETWORK: N00327
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Entry
N00327 Network
Name
Mutation-inactivated PRKAR1A to CRHR-PKA-ACTH signaling pathway
Definition
CRH -> CRHR -> GNAS -> ADCY -> cAMP -> (PRKAR1A*+PRKACA) -> CREB -> ACTH
Expanded
1392 -> (1394,
1395) -> 2778 -> (107,
108,
109,
196883,
111,
112,
113,
114,
115) -> C00575 -> (5573v1+5566) -> (10488,
1385,
1386,
1388,
148327,
468,
64764,
84699,
90993,
9586) -> 5443
Class
nt06360
Cushing syndrome
Type
Variant
Pathway
hsa04934
Cushing syndrome
Disease
H01431
Cushing syndrome
H01102
Pituitary adenomas
Gene
1392
CRH; corticotropin releasing hormone
1394
CRHR1; corticotropin releasing hormone receptor 1
1395
CRHR2; corticotropin releasing hormone receptor 2
2778
GNAS; GNAS complex locus
107
ADCY1; adenylate cyclase 1
108
ADCY2; adenylate cyclase 2
109
ADCY3; adenylate cyclase 3
196883
ADCY4; adenylate cyclase 4
111
ADCY5; adenylate cyclase 5
112
ADCY6; adenylate cyclase 6
113
ADCY7; adenylate cyclase 7
114
ADCY8; adenylate cyclase 8
115
ADCY9; adenylate cyclase 9
5573
PRKAR1A; protein kinase cAMP-dependent type I regulatory subunit alpha
5566
PRKACA; protein kinase cAMP-activated catalytic subunit alpha
10488
CREB3; cAMP responsive element binding protein 3
1385
CREB1; cAMP responsive element binding protein 1
1386
ATF2; activating transcription factor 2
1388
ATF6B; activating transcription factor 6 beta
148327
CREB3L4; cAMP responsive element binding protein 3 like 4
468
ATF4; activating transcription factor 4
64764
CREB3L2; cAMP responsive element binding protein 3 like 2
84699
CREB3L3; cAMP responsive element binding protein 3 like 3
90993
CREB3L1; cAMP responsive element binding protein 3 like 1
9586
CREB5; cAMP responsive element binding protein 5
5443
POMC; proopiomelanocortin
Variant
5573v1
(PRKAR1A*) PRKAR1A mutation
Metabolite
C00575
3',5'-Cyclic AMP
Reference
PMID:
12829237
Authors
Stergiopoulos SG, Stratakis CA
Title
Human tumors associated with Carney complex and germline PRKAR1A mutations: a protein kinase A disease!
Journal
FEBS Lett 546:59-64 (2003)
DOI:
10.1016/S0014-5793(03)00452-6
Reference
PMID:
25877147
Authors
Fukuoka H, Takahashi Y
Title
The role of genetic and epigenetic changes in pituitary tumorigenesis.
Journal
Neurol Med Chir (Tokyo) 54 Suppl 3:943-57 (2014)
DOI:
10.2176/nmc.suppl.2014-3
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