ライフサイエンスコーパス: lamin C

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1 lusively non-farnesylated prelamin A (and no lamin C).

2 s cardiomyopathy (at least in the absence of lamin C).

3 d by shifting the output of LMNA more toward lamin C.

4 members with a defect in the tail domain of lamin C.

5 The alternatively spliced products of LMNA, lamin C and prelamin A (the precursor to lamin A), are p

6 r site in exon 11 of LMNA (the gene encoding lamin C and prelamin A).

7 rmate Lmna HG/+ mice (which produce lamin A, lamin C, and progerin).

8 where nuclear-envelope proteins lamin A and lamin C are encoded by the LMNA (lamin A/C) gene.

9 in the nuclear envelope proteins lamin A and lamin C as the cause of a diverse group of human disease

10 The consequences of Lamin C association at a gene were tested directly by ta

11 pathy could not be ascribed to an absence of lamin C because mice expressing an otherwise identical k

12 e earlier observation that IgG against human lamin C binds to the NTPase in immunoblots.

13 Lamin A and lamin C, both products of Lmna, are key components of th

14 Neurons in the brain produce lamin C but almost no lamin A, a consequence of the remo

15 n C-only mice (Lmna(LCO/LCO)), which produce lamin C but no lamin A or prelamin A (the precursor to l

16 na LCO allele (a mutant allele that produces lamin C but no lamin A).

17 equence has some similarity to that of human lamin C, but not high enough to account for the earlier

18 des all A-type lamins, including lamin A and lamin C, cause a variety of tissue-specific degenerative

19 -specific expression of wild type Drosophila Lamin C caused no overt phenotype.

20 This exon does not comprise part of the lamin C coding region.

21 Association of Lamin C correlated with localization of the reporter gen

22 form of Lamin C lacking the N-terminal head (Lamin C DeltaN) caused muscle defects and semi-lethality

23 t a gene were tested directly by targeting a Lamin C DNA-binding domain fusion protein upstream of a

24 Lamin C exposure after wounding was most likely the cons

25 s showed that prelamin A expression, but not lamin C expression, is down-regulated by a brain-specifi

26 p57, ERp5, and HSP47), and nuclear proteins (lamin C, heterogeneous nuclear ribonucleoprotein F, and

27 and immunohistochemistry studies showed that lamin C is abundant in the mouse brain, whereas lamin A

28 of the 2 different Lmna products lamin A and lamin C is unclear, although several reports have sugges

29 e-specific expression of a truncated form of Lamin C lacking the N-terminal head (Lamin C DeltaN) cau

30 ut not carboxymethylation (by Icmt), whereas lamin C lamina stability is not affected by the loss of

31 the mouse, a deficiency in both lamin A and lamin C leads to slow growth, muscle weakness, and death

32 Lamin C-only cells had slightly abnormal nuclear shape a

33 Here we report the development of lamin C-only mice (Lmna(LCO/LCO)), which produce lamin C

34 s A and C, cells lacking only lamin A (i.e. "lamin C-only" cells), cells lacking wild-type lamin B1,

35 Reintroduction of lamin A, lamin C, or pRB restores p16(ink4a)-responsiveness to Lm

36 tisense oligonucleotide (ASO) that increased lamin C production at the expense of prelamin A when tra

37 of the nuclear-envelope proteins lamin A and lamin C selectively cause dilated cardiomyopathy with co

38 We conclude that lamin C synthesis is dispensable in mice and that the fa

39 domain of the lamin A/C gene, and one in the lamin C tail domain.

40 strate that the Ig-fold motif located in the lamin C terminus binds directly to proliferating cell nu

41 tations in LMNA (the gene for prelamin A and lamin C) that cause particular muscular dystrophy, cardi

42 tations in LMNA (the gene for prelamin A and lamin C) that result in the deletion of 50 aa within pre

43 sed to express wild-type and mutant forms of Lamin C (the presumed Drosophila A-type lamin), in an ot

44 , for example in the targeting of emerin and lamin C to the nuclear envelope.

45 Lamin C transcripts are present at high levels in the br

46 Lmna HG/LCO mice (which produce progerin and lamin C) with littermate Lmna HG/+ mice (which produce l

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