PMID- 9144232
OWN - NLM
STAT- MEDLINE
DCOM- 19970605
LR  - 20190501
IS  - 0027-8424 (Print)
IS  - 1091-6490 (Electronic)
IS  - 0027-8424 (Linking)
VI  - 94
IP  - 10
DP  - 1997 May 13
TI  - Acute leukemia with promyelocytic features in PML/RARalpha transgenic mice.
PG  - 5302-7
AB  - Acute promyelocytic leukemia (APL) is associated with reciprocal chromosomal 
      translocations involving the retinoic acid receptor alpha (RARalpha) locus on 
      chromosome 17. In the majority of cases, RARalpha translocates and fuses with the 
      promyelocytic leukemia (PML) gene located on chromosome 15. The resulting fusion 
      genes encode the two structurally unique PML/RARalpha and RARalpha/PML fusion 
      proteins as well as aberrant PML gene products, the respective pathogenetic roles 
      of which have not been elucidated. We have generated transgenic mice in which the 
      PML/RARalpha fusion protein is specifically expressed in the 
      myeloid-promyelocytic lineage. During their first year of life, all the 
      PML/RARalpha transgenic mice have an abnormal hematopoiesis that can best be 
      described as a myeloproliferative disorder. Between 12 and 14 months of age, 10% 
      of them develop a form of acute leukemia with a differentiation block at the 
      promyelocytic stage that closely mimics human APL even in its response to 
      retinoic acid. Our results are conclusive in vivo evidence that PML/RARalpha 
      plays a crucial role in the pathogenesis of APL.
FAU - He, L Z
AU  - He LZ
AD  - Department of Human Genetics, Memorial Sloan-Kettering Cancer Center, Molecular 
      Biology and Cell Biology Programs, Sloan-Kettering Institute, 1275 York Avenue, 
      New York, NY, 10021, USA.
FAU - Tribioli, C
AU  - Tribioli C
FAU - Rivi, R
AU  - Rivi R
FAU - Peruzzi, D
AU  - Peruzzi D
FAU - Pelicci, P G
AU  - Pelicci PG
FAU - Soares, V
AU  - Soares V
FAU - Cattoretti, G
AU  - Cattoretti G
FAU - Pandolfi, P P
AU  - Pandolfi PP
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Proc Natl Acad Sci U S A
JT  - Proceedings of the National Academy of Sciences of the United States of America
JID - 7505876
RN  - 0 (DNA Primers)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (Nuclear Proteins)
RN  - 0 (Pml protein, mouse)
RN  - 0 (Promyelocytic Leukemia Protein)
RN  - 0 (RARA protein, human)
RN  - 0 (Rara protein, mouse)
RN  - 0 (Receptors, Retinoic Acid)
RN  - 0 (Recombinant Fusion Proteins)
RN  - 0 (Retinoic Acid Receptor alpha)
RN  - 0 (Transcription Factors)
RN  - 0 (Tumor Suppressor Proteins)
RN  - 143220-95-5 (PML protein, human)
RN  - 5688UTC01R (Tretinoin)
SB  - IM
MH  - Aging
MH  - Animals
MH  - Blood Cell Count
MH  - Bone Marrow/pathology
MH  - Cell Differentiation/drug effects
MH  - Chromosomes, Human, Pair 17
MH  - DNA Primers
MH  - Hematopoiesis
MH  - Hematopoietic Stem Cells/cytology/drug effects/pathology
MH  - Humans
MH  - Leukemia, Promyelocytic, Acute/blood/*genetics/pathology
MH  - Lymphocytes/cytology/drug effects/pathology
MH  - Mice
MH  - Mice, Transgenic
MH  - Myeloproliferative Disorders/genetics/physiopathology
MH  - *Neoplasm Proteins
MH  - *Nuclear Proteins
MH  - Polymerase Chain Reaction
MH  - Promyelocytic Leukemia Protein
MH  - Receptors, Retinoic Acid/biosynthesis/*genetics
MH  - Recombinant Fusion Proteins/*biosynthesis
MH  - Reference Values
MH  - Retinoic Acid Receptor alpha
MH  - Spleen/pathology
MH  - Transcription Factors/biosynthesis/*genetics
MH  - Translocation, Genetic
MH  - Tretinoin/pharmacology
MH  - Tumor Suppressor Proteins
PMC - PMC24673
EDAT- 1997/05/13 00:00
MHDA- 1997/05/13 00:01
PMCR- 1997/11/13
CRDT- 1997/05/13 00:00
PHST- 1997/05/13 00:00 [pubmed]
PHST- 1997/05/13 00:01 [medline]
PHST- 1997/05/13 00:00 [entrez]
PHST- 1997/11/13 00:00 [pmc-release]
AID - 0631 [pii]
AID - 10.1073/pnas.94.10.5302 [doi]
PST - ppublish
SO  - Proc Natl Acad Sci U S A. 1997 May 13;94(10):5302-7. doi: 
      10.1073/pnas.94.10.5302.