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[95] Bateman JF, Chan D, Mascara T, Rogers JG, Cole WG. Collagen
defects in lethal perinatal osteogenesis imperfecta. Biochem J
1986;240(3):699-708.
[96] Brenner RE, Vetter U, Nerlich A, Worsdorfer O, Teller WM,
Muller PK. Osteogenesis imperfecta: insuficient collagen syn-
thesis in early childhood as evidenced by analysis of compact
bone and ibroblast cultures. Eur J Clin Invest 1989;19(2):159-66.
[97] Brenner RE, Vetter U, Nerlich A, Worsdorfer O, Teller WM,
Muller PK. Altered collagen metabolism in osteogenesis imper-
fecta ibroblasts: a study on 33 patients with diverse forms. Eur
J Clin Invest 1990;20(1):8-14.
[98] Morike M, Brenner RE, Bushart GB, Teller WM, Vetter U.
Collagen metabolism in cultured osteoblasts from osteogenesis
imperfecta patients. Biochem J 1992;286(Pt 1):73-7.
[99] de Wet WJ, Chu ML, Prockop DJ. The mRNAs for the pro-
alpha 1(I) and pro-alpha 2(I) chains of type I procollagen are
translated at the same rate in normal human ibroblasts and in
ibroblasts from two variants of osteogenesis imperfecta with
altered steady state ratios of the two mRNAs. J Biol Chem
1983;258(23):14385-89.
of cultured dermal ibroblasts in osteogenesis imperfecta.
Connect Tissue Res 1993;29(1):41-9.
[111]
Bateman JF, Golub SB. Deposition and selective degradation of
structurally-abnormal type I collagen in a collagen matrix pro-
duced by osteogenesis imperfecta ibroblasts in vitro. Matrix
Biol 1994;14(3):251-62.
[112]
Valli M, Rossi A, Forlino A, Tenni R, Cetta G. Extracellular matrix
deposition in cultured dermal ibroblasts from four probands
affected by osteogenesis imperfecta. Matrix 1993;13(4):275-80.
[113]
Kapoor R, Bourier S, Prehm P. Glycosaminoglycan synthesis
in skin ibroblasts from patients with osteogenesis imperfecta.
FEBS Lett 1983;152(2):183-6.
[114]
De Luca G, Tira ME, Rindi S, Salvini R, Cetta G, Castellani AA.
Proteoglycan modiications in cultured osteogenesis imperfecta
skin ibroblasts. Ital J Biochem 1984;33(6):403-12.
[115]
Dyne KM, Valli M, Forlino A, Mottes M, Kresse H, Cetta G.
Deicient expression of the small proteoglycan decorin in a
case of severe/lethal osteogenesis imperfecta. Am J Med Genet
1996;63(1):161-6.
[116]
Turakainen H. Altered glycosaminoglycan production in cul-
tured osteogenesis-imperfecta skin ibroblasts. Biochem J
1983;213(1):171-8.
[100]
Chu ML, Rowe D, Nicholls AC, Pope FM, Prockop DJ. Presence
of translatable mRNA for pro alpha 2(I) chains in ibroblasts
from a patient with osteogenesis imperfecta whose type I
collagen does not contain alpha 2(I) chains. Coll Relat Res
1984;4(5):389-94.
[117]
Turakainen H, Larjava H, Saarni H, Penttinen R. Synthesis of
hyaluronic acid and collagen in skin ibroblasts cultured from
patients with osteogenesis imperfecta. Biochim Biophys Acta
1980;628(4):388-97.
[101]
Rowe DW, Shapiro JR, Poirier M, Schlesinger S. Diminished
type I collagen synthesis and reduced alpha 1(I) collagen mes-
senger RNA in cultured ibroblasts from patients with domi-
nantly inherited (type I) osteogenesis imperfecta. J Clin Invest
1985;76(2):604-11.
[118]
Boright AP, Lancaster GA, Scriver CR. Osteogenesis imper-
fecta: a heterogeneous morphologic phenotype in cultured der-
mal ibroblasts. Hum Genet 1984;67(1):29-33.
[119]
Meerson EM, Barer FS, Berezhnyi AP, Prokhorova EV,
Maliavko TP, Chernysheva EV, et al. Morphofunctional aspects
of genetic analysis of osteogenesis imperfecta in cultured skin
ibroblasts. Biull Eksp Biol Med 1992;114(7):86-8.
[102]
Genovese C, Rowe D. Analysis of cytoplasmic and nuclear
messenger RNA in ibroblasts from patients with type I osteo-
genesis imperfecta. Methods Enzymol 1987;145:223-35.
[103]
Peltonen L, Palotie A, Hayashi T, Prockop DJ. Thermal stability
of type I and type III procollagens from normal human ibro-
blasts and from a patient with osteogenesis imperfecta. Proc
Natl Acad Sci USA 1980;77(1):162-6.
[120]
Baron R, Gertner JM, Lang R, Vignery A. Increased bone turn-
over with decreased bone formation by osteoblasts in children
with osteogenesis imperfecta tarda. Pediatr Res 1983;17(3):204-7.
[121]
Stoss H, Pontz BF, Pesch HJ, Ott R. Heterogeneity of osteogene-
sis imperfecta. Biochemical and morphological indings in a case
of type III according to Sillence. Eur J Pediatr 1986;145(1-2):34-9.
[104]
Sippola M, Kaffe S, Prockop DJ. A heterozygous defect for
structurally altered pro-alpha 2 chain of type I procolla-
gen in a mild variant of osteogenesis imperfecta. The altered
structure decreases the thermal stability of procollagen and
makes it resistant to procollagen N-proteinase. J Biol Chem
1984;259(22):14094-100.
[122]
Cassella JP, Stamp TC, Ali SY. A morphological and ultrastruc-
tural study of bone in osteogenesis imperfecta. Calcif Tissue Int
1996;58(3):155-65.
[123]
Iwamoto J, Takeda T, Ichimura S. Increased bone resorption
with decreased activity and increased recruitment of osteo-
blasts in osteogenesis imperfecta type I. J Bone Miner Metab
2002;20(3):174-9.
[105]
Bonaventure J, Cohen-Solal L, Lasselin C, Allain JC, Maroteaux P.
Abnormal procollagen synthesis in ibroblasts from three patients
of the same family with a severe form of osteogenesis imperfecta
(type III). Biochim Biophys Acta 1986;889(1):23-34.
[124]
Chipman SD, Shapiro JR, McKinstry MB, Stover ML, Branson
P, Rowe DW. Expression of mutant alpha (I)-procollagen in
osteoblast and ibroblast cultures from a proband with osteo-
genesis imperfecta type IV. J Bone Miner Res 1992;7(7):793-805.
[106]
Tenni R, Cetta G, Dyne K, Rossi A, Quacci D, Lenzi L, et  al.
Type I procollagen in the severe non-lethal form of osteogene-
sis imperfecta. Defective pro-alpha 1(I) chains in a patient with
abnormal proteoglycan metabolism and mineral deposits in
the dermis. Hum Genet 1988;79(3):245-50.
[125]
Fedarko NS, Moerike M, Brenner R, Robey PG, Vetter U.
Extracellular matrix formation by osteoblasts from patients with
osteogenesis imperfecta. J Bone Miner Res 1992;7(8):921-30.
[107]
Tajima S, Takehana M, Azuma N. Production of overmodi-
ied type I procollagen in a case of osteogenesis imperfecta.
J Dermatol 1994;21(4):219-22.
[126]
Galicka A, Wolczynski S, Gindzienski A. Comparative studies
of osteoblast and ibroblast type I collagen in a patient with
osteogenesis imperfecta type IV. J Pathol 2002;196(2):235-7.
[108]
Rao VH, Steinmann B, de Wet W, Hollister DW. Decreased ther-
mal denaturation temperature of osteogenesis imperfecta mutant
collagen is independent of post-translational overmodiications
of lysine and hydroxylysine. J Biol Chem 1989;264(3):1793-8.
[127]
Sarafova AP, Choi H, Forlino A, Gajko A, Cabral WA, Tosi L,
et  al. Three novel type I collagen mutations in osteogenesis
imperfecta type IV probands are associated with discrepancies
between electrophoretic migration of osteoblast and ibroblast
collagen. Hum Mutat 1998;11(5):395-403.
[109]
Steinmann BU, Martin GR, Baum BI. Crystal RG. Synthesis
and degradation of collagen by skin ibroblasts from controls
and from patients with osteogenesis imperfecta. FEBS Lett
1979;101(2):269-72.
[128]
Morike M, Windsheimer E, Brenner R, Nerlich A, Bushart G,
Teller W, et  al. Effects of transforming growth factor beta on
cells derived from bone and callus of patients with osteogen-
esis imperfecta. J Orthop Res 1993;11(4):564-72.
[110]
Cetta G, Rossi A, Tenni R, Valli M, Forlino A, Zanaboni G, et al.
Deposition of mutant type I collagen in the extracellular matrix
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