PMID- 12061838
OWN - NLM
STAT- MEDLINE
DCOM- 20021231
LR  - 20051117
IS  - 1043-4666 (Print)
IS  - 1043-4666 (Linking)
VI  - 17
IP  - 6
DP  - 2002 Mar 21
TI  - Collagens, collagen-binding heat shock protein 47 and transforming growth 
      factor-beta 1 are induced in cicatricial pemphigoid: possible role(s) in dermal 
      fibrosis.
PG  - 311-6
AB  - Cicatricial pemphigoid (CP) is an autoimmune mucocutaneous blistering disease 
      associated with scarring. Heat shock protein 47 (HSP47) is thought to play an 
      important role in fibrogenesis, but its role in skin lesions of cicatricial 
      pemphigoid is not yet known. In the present study, we examined the role of HSP47 
      in dermal fibrosis in cutaneous lesions of a CP patient. Skin biopsies from a 
      patient with CP, and from normal subjects were studied for the expression of 
      HSP47, and interstitial collagens (type I and type III collagens) by 
      immunohistochemistry. Dermal fibroblasts isolated from skin of normal individuals 
      and from fibrotic skin of a CP patient were used to study the expression of 
      HSP47, transforming growth factor beta 1 (TGF-beta 1), type I and type III 
      collagens. Compared to the control skin sections, an increased expression of 
      HSP47 was associated with an increased deposition of interstitial collagens in 
      the fibrotic skin section of the CP patient. Similarly, in contrast to control 
      dermal fibroblasts, the fibroblasts isolated and cultured from fibrotic skin of 
      the CP patient, and grown in vitro, exhibited increased expression of HSP47, type 
      I and type III collagens. Furthermore, compared to the normal control 
      fibroblasts, an increased expression of TGF-beta 1 was detected in the dermal 
      fibroblasts isolated from fibrotic skin of the CP patient. When dermal 
      fibroblasts were treated with various concentrations of TGF-beta 1 (6.25, 12.5, 
      25, 50 and 100 ng/ml for 24 h), it induced the expression of both type I collagen 
      and HSP47, as determined by quantitative real-time PCR. In conclusion, the 
      expression of TGF-beta 1, HSP47, type I collagen and type III collagen was 
      up-regulated in the fibrotic skin of CP patient, and a complex interaction of 
      these molecules may initiate and propagate the fibrotic cascade in the skin of CP 
      patients.
CI  - Copyright 2002 Elsevier Science Ltd. All rights reserved.
FAU - Razzaque, M S
AU  - Razzaque MS
AD  - Department of Dermatology, Harvard Medical School, Boston, MA, USA.
FAU - Ahmed, A R
AU  - Ahmed AR
LA  - eng
PT  - Journal Article
PL  - England
TA  - Cytokine
JT  - Cytokine
JID - 9005353
RN  - 0 (DNA Primers)
RN  - 0 (HSP47 Heat-Shock Proteins)
RN  - 0 (Heat-Shock Proteins)
RN  - 0 (Recombinant Proteins)
RN  - 0 (SERPINH1 protein, human)
RN  - 0 (Transforming Growth Factor beta)
RN  - 9007-34-5 (Collagen)
SB  - IM
MH  - Base Sequence
MH  - Collagen/*genetics
MH  - DNA Primers
MH  - Fibroblasts/pathology
MH  - Fibrosis
MH  - Gene Expression Regulation/drug effects
MH  - HSP47 Heat-Shock Proteins
MH  - Heat-Shock Proteins/*genetics
MH  - Humans
MH  - Pemphigoid, Benign Mucous Membrane/*metabolism/physiopathology
MH  - Polymerase Chain Reaction/methods
MH  - Recombinant Proteins/pharmacology
MH  - Reverse Transcriptase Polymerase Chain Reaction
MH  - Skin/drug effects/*pathology
MH  - Transforming Growth Factor beta/*genetics/pharmacology
EDAT- 2002/06/14 10:00
MHDA- 2003/01/01 04:00
CRDT- 2002/06/14 10:00
PHST- 2002/06/14 10:00 [pubmed]
PHST- 2003/01/01 04:00 [medline]
PHST- 2002/06/14 10:00 [entrez]
AID - S1043466602910203 [pii]
AID - 10.1006/cyto.2002.1020 [doi]
PST - ppublish
SO  - Cytokine. 2002 Mar 21;17(6):311-6. doi: 10.1006/cyto.2002.1020.