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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Nat+Commun
2016 ; 7
(ä): 10087
Nephropedia Template TP
gab.com Text
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English Wikipedia
Anaplastic large cell lymphoma arises in thymocytes and requires transient TCR
expression for thymic egress
#MMPMID26753883
Malcolm TI
; Villarese P
; Fairbairn CJ
; Lamant L
; Trinquand A
; Hook CE
; Burke GA
; Brugières L
; Hughes K
; Payet D
; Merkel O
; Schiefer AI
; Ashankyty I
; Mian S
; Wasik M
; Turner M
; Kenner L
; Asnafi V
; Macintyre E
; Turner SD
Nat Commun
2016[Jan]; 7
(ä): 10087
PMID26753883
show ga
Anaplastic large cell lymphoma (ALCL) is a peripheral T-cell lymphoma presenting
mostly in children and young adults. The natural progression of this disease is
largely unknown as is the identity of its true cell of origin. Here we present a
model of peripheral ALCL pathogenesis where the malignancy is initiated in early
thymocytes, before T-cell receptor (TCR) ?-rearrangement, which is bypassed in
CD4/NPM-ALK transgenic mice following Notch1 expression. However, we find that a
TCR is required for thymic egress and development of peripheral murine tumours,
yet this TCR must be downregulated for T-cell lymphomagenesis. In keeping with
this, clonal TCR rearrangements in human ALCL are predominantly in-frame, but
often aberrant, with clonal TCR? but no comparable clonal TCR? rearrangement,
yielding events that would not normally be permissive for survival during thymic
development. Children affected by ALCL may thus harbour thymic
lymphoma-initiating cells capable of seeding relapse after chemotherapy.