KEGG   DISEASE: Adult T-cell leukemiaHelp
Entry
H00009                      Disease                                

Name
Adult T-cell leukemia
Description
Adult T-cell leukemia (ATL) is one of the most aggressive hematologic malignancies and is caused by human T-cell leukemia virus type 1 (HTLV-1). The HTLV-1 Tax protein has been demonstrated to be the oncogenic protein of the virus. Tax may contribute to the process of carcinogenesis by a variety of mechanisms, including upregulating the expression of cellular genes involved in T cell growth and proliferation, including IL-2, IL-2R-alpha, and IL-15. However, ATL cells do not always need Tax expression in the later stage of leukemogenesis. Genetic and epigenetic changes should be implicated in such multistep leukemogenesis. Regarding genetic changes, mutation of p53, and deletion of p16 have been reported in ATL. Mutations of Fas gene are also reported in patients with ATL cells. However, such genetic changes were not frequently detected. In this regard, epigenetic change of p16/INK4A gene was more frequent in ATL cells, and accumulated according to the disease progression. This finding suggests that epigenetic change, including DNA methylation, plays an important role in the leukemogenesis of ATL.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00009  Adult T-cell leukemia
Infectious diseases [BR:br08401]
 Viral infections
  Infections caused by ssRNA-RT viruses
   H00009  Adult T-cell leukemia
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature T-cell or NK-cell neoplasms
    2A90  Mature T-cell lymphoma, specified types, nodal or systemic
     H00009  Adult T-cell leukemia
Tumor markers [br08442.html]
 H00009
BRITE hierarchy
Pathway
hsa05166  Human T-cell leukemia virus 1 infection
hsa05203  Viral carcinogenesis
Network
N00221  HTLV-1 Tax to spindle assembly checkpoint signaling
N00222  HTLV-1 Tax to spindle assembly checkpoint signaling
N00489  HTLV-1 p30II to c-myc-mediated transcription
N00490  HTLV-1 p12 to calcineurin signaling pathway
N00491  HTLV-1 p12 to Jak-STAT signaling pathway
N00492  HTLV-1 p12 to antigen processing and presentation by MHC class I molecules
N00494  HTLV-1 Tax to p16-cell cycle G1/S
N00495  HTLV-1 Tax to p15-cell cycle G1/S
N00497  HTLV-1 Tax to p21-cell cycle G1/S
N00498  HTLV-1 Tax to p21-cell cycle G1/S
N00500  HTLV-1 Tax to p21-cell cycle G2/M
N00501  HTLV-1 Tax to EGFR-PI3K-NFKB signaling pathway
N00502  HTLV-1 Tax to PTEN-PIP3-AKT signaling pathway
N00503  HTLV-1 Tax to TNF-JNK signaling pathway
N00504  HTLV-1 Tax to NFKB signaling pathway
N00506  HTLV-1 Tax to CD40-NFKB signaling pathway
N00507  HTLV-1 Tax to TGF-beta signaling pathway
N00508  HTLV-1 Tax to NFY-mediated transcription
N00509  HTLV-1 Tax to SRF-mediated transcription
N00510  HTLV-1 Tax to CREB-mediated transcription
N00511  HTLV-1 Tax to E47-mediated transcription
N00512  HTLV-1 Tax to c-myc-mediated transcription
 » show all
Gene
p53 (mutation) [HSA:7157] [KO:K04451]
p16/INK4A (deletion, promoter methylation) [HSA:1029] [KO:K06621]
Fas (mutation) [HSA:355] [KO:K04390]
Pathogen
Human T-lymphotropic virus 1 [GN:T40003]
Comment
ICD-O: 9827/3, Tumor type: Adult T-cell leukaemia
Other DBs
ICD-11: 2A90.5
ICD-10: C91.5
MeSH: D015459
Reference
  Authors
Matsuoka M
  Title
Human T-cell leukemia virus type I and adult T-cell leukemia.
  Journal
Oncogene 22:5131-40 (2003)
DOI:10.1038/sj.onc.1206551
Reference
  Authors
Yasunaga J, Matsuoka M
  Title
Leukemogenesis of adult T-cell leukemia.
  Journal
Int J Hematol 78:312-20 (2003)
DOI:10.1007/BF02983555
Reference
PMID:10190897 (gene, tumor type)
  Authors
Maeda T, Yamada Y, Moriuchi R, Sugahara K, Tsuruda K, Joh T, Atogami S, Tsukasaki K, Tomonaga M, Kamihira S.
  Title
Fas gene mutation in the progression of adult T cell leukemia.
  Journal
J Exp Med 189:1063-71 (1999)
DOI:10.1084/jem.189.7.1063
Reference
PMID:17387298 (carcinogen)
  Authors
Yasunaga J, Matsuoka M.
  Title
Human T-cell leukemia virus type I induces adult T-cell leukemia: from clinical aspects to molecular mechanisms.
  Journal
Cancer Control 14:133-40 (2007)
DOI:10.1177/107327480701400206
Reference
PMID:16362996 (carcinogen)
  Authors
Jarrett RF.
  Title
Viruses and lymphoma/leukaemia.
  Journal
J Pathol 208:176-86 (2006)
DOI:10.1002/path.1905
LinkDB All DBs

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