ライフサイエンスコーパス: ALR

1 ALR concentrations were then determined in intact livers

2 ALR forms large amounts of neutral semiquinone during ae

3 ALR has trophic effects on regenerating liver.

4 ALR in the various liver cells was localized with immuno

5 ALR is a cellular process controlled by mTOR which regen

6 ALR is a convenient and useful regression technique for

7 ALR islets were found to be remarkably resistant to two

8 ALR mice are unusual in their high constitutive expressi

9 ALR resistance to diabetogenic immune systems was determ

10 ALR was delivered from day 1 through day 14, in doses of

11 ALR-L-KO mice provide a useful model for investigating t

12 on of ALRs, we generated mice lacking all 13 ALR genes.

13 e column (BCR) and an internal loop airlift (ALR) bioreactors of 2.3 L for the abatement of N2O from

14 t of impaired lysosomal tubulation alongside ALR activation is massive cell death.

15 ed mice with liver-specific deletion of ALR (ALR-L-KO) using the albumin-Cre/LoxP system.

16 itive genetic variance but also that AFR and ALR are positively genetically correlated.

17 In addition, hepatic ALR and ALR mRNA were assayed with Western blotting and reverse-

18 and the reciprocal with NOD nuclear DNA and ALR mtDNA (NOD.mt(ALR)).

19 gene, 9GL, with similarity to yeast ERV1 and ALR genes.

20 ALS/Lt and ALR/Lt are inbred mouse strains selected for susceptibil

21 issues were collected from ALR-L-KO mice and ALR(floxed/floxed) mice (controls) and analyzed by histo

22 neage leukemia 4 (MLL4; also called MLL2 and ALR) enzymatically generates trimethylated histone H3 Ly

23 he close MHC relatedness between the NOD and ALR strains (H2-Kd and H2-Ag7 identical) allowed us to e

24 rary to previous belief, the ALR peptide and ALR mRNA were present in comparable concentrations in th

25 rucial for endosome-lysosome trafficking and ALR, accumulated in GCase deficient cells, supporting th

26 NLRs and ALRs engage caspase-1, in most cases requiring the adapt

27 Unlike MLL1, MLL2 (also known as ALR/MLL4) and its homolog MLL3 are not well-understood.

28 Because ALR-1 and similar proteins (Drosophila Aristaless and hu

29 tion adversely affects the stability of both ALR forms: e.g., by decreasing the melting temperature b

30 nerated in NOD and C3H/HeJ islets but not by ALR islets.

31 lceramide homeostasis which could compromise ALR.

32 In contrast, ALR/Lt islets remained viable and retained glucose-stimu

33 3 is not sufficient for TRN differentiation; ALR-1 provides a second positive feedback loop that incr

34 e R194H mutation on the stability of dimeric ALR and complement the in vivo observations of Di Fonzo

35 ulfide, a helix-rich domain, and then an Erv/ALR domain.

36 ace, and a larger protein containing the ERV/ALR domain, quiescin-sulfhydryl oxidase (QSOX).

37 domain and the proximal disulfide of the Erv/ALR domain.

38 The FAD prosthetic group of the ERV/ALR family of sulfhydryl oxidases is housed at the mouth

39 redox properties between members of the ERV/ALR family of sulfhydryl oxidases provides insights into

40 ulfhydryl oxidases and is related to the ERV/ALR family of sulfhydryl oxidases.

41 irus in which the E10R gene encoding an ERV1/ALR family protein was repressed, the disulfide bonds of

42 ial disulfide relay system of Mia40 and Erv1/ALR facilitates import of the small translocase of the i

43 Multiple alignments of ERV1/ALR proteins indicated an invariant C-X-X-C motif, but n

44 sulfhydryl:cytochrome c oxidoreductase Erv1/ALR are essential for oxidative protein import into the

45 Proteins of the ERV1/ALR family are encoded by all eukaryotes and cytoplasmic

46 By extension, the ERV1/ALR family may represent a ubiquitous class of cellular

47 pstream component, E10R, belongs to the ERV1/ALR family of FAD-containing sulfhydryl oxidases that us

48 vaccinia virus protein belonging to the ERV1/ALR family, has a redox function and is required for vir

49 cells from all Kd-expressing strains except ALR.

50 y express the auxiliary transcription factor ALR-1 (Aristaless related), which ensures, but does not

51 n the gene encoding the transcription factor ALR-1 in Caenorhabditis elegans result in variable touch

52 e binding sites of 22 transcription factors (ALR-1, BLMP-1, CEH-14, CEH-30, EGL-27, EGL-5, ELT-3, EOR

53 The flavoprotein ALR receives two electrons per subunit from Mia40, which

54 bout a 100-fold better electron acceptor for ALR than oxygen when DTT is the reducing substrate.

55 ated cells, suggesting an important role for ALR in hESC homeostasis.

56 results indicate a cell autonomous role for ALR-1 in maintaining cell shape.

57 Furthermore, we demonstrate a role for ALR-1 in the proper morphogenesis of the anterior hypode

58 n a significantly greater proportion of FP + ALR-treated recipients than those animals treated with v

59 Liver tissues were collected from ALR-L-KO mice and ALR(floxed/floxed) mice (controls) and

60 vivo, ALS/Lt islets were distinguished from ALR/Lt islets by more extensive necrotic changes.

61 d the mechanism whereby isolated islets from ALR mice resisted proinflammatory stress mediated by com

62 of hydrogen peroxide and NO than islets from ALR mice.

63 A dominant genetic trait from ALR/Lt controlling this unusual AL resistance was indica

64 We have cloned a novel human gene, ALR, which encodes a gigantic 5262 amino acid long prote

65 Hence, ALR/Lt islets resist cytokine-induced diabetogenic stres

66 In addition, hepatic ALR and ALR mRNA were assayed with Western blotting and

67 further unexpected finding was that hepatic ALR levels decreased for 12 hours after 70% hepatectomy

68 However, ALR.mt(NOD) mitochondria supported by either complex I o

69 et al. described an R194H mutation of human ALR that led to cataract, progressive muscle hypotonia,

70 est that, contrary to a recent report, human ALR is a disulfide-bridged dimer (linked via C15-C124) w

71 ation, and functions of the murine and human ALRs and identify novel activators of STING-dependent IF

72 We illustrate ALR with an application to caries aggregation using a da

73 Nuclear and mitochondrial genomes combine in ALR/Lt mice to produce systemically elevated defenses ag

74 vels of steatosis and apoptosis decreased in ALR-L-KO mice, and numbers of ALR-expressing cells incre

75 ied as the rare H2-Ddx recently described in ALR/Lt, another NOD-related strain.

76 on of autolysosomes, reflecting a failure in ALR.

77 ted antioxidant defenses previously found in ALR/Lt pancreas were extended to isolated islets, which

78 ase in NOD-Rag and C3H/HeJ islets but not in ALR islets.

79 e model for the electron-transfer pathway in ALR.

80 in localize the heightened ROS production in ALR.mt(NOD) to complex III and identified complex I as t

81 We have recently shown that resistance in ALR/Lt correlated with constitutively elevated systemic

82 rom all mt-Nd2(c)-encoding strains including ALR.mt(NOD), non-obese diabetic (NOD), and C57BL/6 (B6).

83 Investigating ALR-1 functions in C. elegans may yield insights into th

84 clone or by chimerizing lethally irradiated ALR or reciprocal (ALR x NOD)F1 recipients with NOD bone

85 in and noticed that a subset of MLL2 (KMT2D, ALR, MLL4)-associated Kabuki syndrome missense mutations

86 some individuals have earlier AFR and later ALR than expected.

87 itment of the inflammasome adaptor ASC links ALRs to the activation of caspase-1.

88 d physiological substrates of short and long ALR forms.

89 perform a phylogenetic analysis of mammalian ALRs, revealing a remarkable diversification of these re

90 one lysine methyltransferases (HKMTs), MLL2 (ALR), forming a complex that methylates lysine 4 of hist

91 of mammalian Mixed Lineage Leukemia 2 (MLL2/ALR), a core component of COMPASS-like nuclear receptor

92 Moreover, ALR dysfunction was accompanied by impairment of macroau

93 ntribute to each of these pathways, but most ALRs remain uncharacterized.

94 ed from either the NOD (NIT-1) or the NOD.mt(ALR) (NIT-4) were also created to compare their suscepti

95 compared with both parental strains, NOD.mt(ALR) or C57BL/6 controls.

96 nt (ALR)/Lt-derived mt-Nd2(a) allele (NOD.mt(ALR)) were created and compared with standard NOD (carry

97 l with NOD nuclear DNA and ALR mtDNA (NOD.mt(ALR)).

98 ted: one with ALR nuclear DNA and NOD mtDNA (ALR.mt(NOD)) and the reciprocal with NOD nuclear DNA and

99 Here, we identify five novel murine ALRs and perform a phylogenetic analysis of mammalian AL

100 Here, we identified a non-NLR/ALR human protein that stimulates inflammasome assembly:

101 interfere with the interaction between NLRPs/ALRs and ASC to prevent nucleation of ASC and therefore

102 inhibitor of DNA virus-induced activation of ALR inflammasomes in vivo.

103 the dithiothreitol (DTT) oxidase activity of ALR.

104 a40 and requires tightly balanced amounts of ALR.

105 The analysis of ALR expression showed that its approximately 18 kb long

106 veloped mice with liver-specific deletion of ALR (ALR-L-KO) using the albumin-Cre/LoxP system.

107 created mice with liver-specific deletion of ALR to study its function.

108 veloped mice with liver-specific deletion of ALR, and showed that it is required for mitochondrial fu

109 In addition to the cytokine form of ALR (sfALR) that circulates in serum, a longer form, lfA

110 er of both the short, cytokine-like, form of ALR (sfALR), and a longer form (lfALR) which resides in

111 free radical stress extended to the level of ALR/Lt pancreatic islets.

112 ers of ALR-L-KO mice contained low levels of ALR and adenosine triphosphate (ATP); they had reduced m

113 Hepatic levels of ALR were also low in ob/ob mice and alcohol-fed mice wit

114 Levels of ALR were lower in liver tissues from patients with advan

115 Two weeks after birth, livers of ALR-L-KO mice contained low levels of ALR and adenosine

116 ase subunit ATP5G1 were reduced in livers of ALR-L-KO mice, indicating defects in mitochondrial fatty

117 s decreased in ALR-L-KO mice, and numbers of ALR-expressing cells increased, along with ATP levels.

118 mphasize the importance of the regulation of ALR inflammasomes in monocytes and macrophages.

119 intaining homeostasis, but the regulation of ALR inflammasomes is poorly understood.

120 nerate neoantigens; therefore, resistance of ALR islets to nitrotyrosine formation may, in part, expl

121 In this work, we investigate the role of ALR-1, the Caenorhabditis elegans aristaless orthologue,

122 nown about the cellular/subcellular sites of ALR synthesis in the liver, or about the release and dis

123 ere assigned by comparing the NMR spectra of ALR bound to oxidized and reduced flavin adenine dinucle

124 To rigorously explore the function of ALRs, we generated mice lacking all 13 ALR genes.

125 and oligomerization, leucine-rich repeat) or ALR (absent in melanoma 2-like receptor) proteins to act

126 the homodimeric FAD-dependent thiol oxidase ALR.

127 arities in structure and expression pattern, ALR is likely to play a similar role to ALL-1 and trx, a

128 III, the Escherichia coli alanine racemase (ALR), is a promiscuous cystathionine beta-lyase (CBL).

129 we raised antibodies in rabbits against rat ALR for development of an enzyme-linked immunosorbent as

130 Although the crystal structure of rat ALR shows a proximal disulfide (C62-C65) poised to inter

131 Certain members of the AIM2-like receptor (ALR) gene family contribute to each of these pathways, b

132 absent in melanoma 2 (AIM2)-like receptors (ALR).

133 osolic DNA is sensed by AIM2-like receptors (ALRs) during bacterial and viral infections and in autoi

134 aining a PYD (NLRPs) or AIM2-like receptors (ALRs), which interact with the PYD- and CARD-containing

135 this pathway, including AIM2-like receptors (ALRs).

136 izing lethally irradiated ALR or reciprocal (ALR x NOD)F1 recipients with NOD bone marrow.

137 The accessory lateral rectus (ALR) EOM was present in all specimens, but was small, in

138 e show that autophagy lysosomal reformation (ALR) is compromised in cells lacking functional GCase.

139 pivotal for autophagic lysosome reformation (ALR), a pathway that generates new lysosomes.

140 s termed autophagosome-lysosome reformation (ALR).

141 ryl oxidase augmenter of liver regeneration (ALR) binds FAD in a helix-rich domain that presents a Cx

142 Augmenter of liver regeneration (ALR) is a hepatotrophic protein originally identified by

143 Augmenter of liver regeneration (ALR) is a newly described polypeptide growth factor foun

144 Augmenter of liver regeneration (ALR) is both a growth factor and a sulfhydryl oxidase th

145 ryl oxidase augmenter of liver regeneration (ALR), and the intracellular glutathione pool.

146 ryl oxidase augmenter of liver regeneration (ALR).

147 Augmenter of liver regeneration (ALR, encoded by GFER) is a widely distributed pleiotropi

148 Augmenter of liver regeneration (ALR; hepatopoietin) is a recently discovered enigmatic f

149 ic factors (augmenter of liver regeneration [ALR], insulin-like growth factor-II [IGF-II], and hepato

150 posable element (LINE), or satellite region (ALR/Alpha) DNA, and three additional clones were near Al

151 such data, alternating logistic regressions (ALR) is a computationally efficient alternative method,

152 oduction (AFR) and age at last reproduction (ALR) in a free-ranging mute swan (Cygnus olor) populatio

153 analysis of outcrosses between T1D-resistant ALR and T1D-susceptible NOD mice.

154 ble NOD mice with the strongly T1D-resistant ALR strain.

155 J mice conplastic for the alloxan resistant (ALR)/Lt-derived mt-Nd2(a) allele (NOD.mt(ALR)) were crea

156 th nuclear genes from the alloxan-resistant (ALR) strain, mt-Nd2(c) increases ROS from complex III.

157 Recombinant GCase treatment reversed ALR inhibition and lysosomal dysfunction.

158 nd stored; the cultured hepatocytes secreted ALR into the medium in a time-dependent fashion.

159 In the meantime, circulating (serum) ALR levels increased up to 12 hours and declined thereaf

160 contrast to islets from other mouse strains, ALR islets expressed constitutively higher glutathione r

161 1Mag and nine Swiss-derived, inbred strains: ALR/LtJ, ALS/LtJ, APN, APS, ICR/HaRos, NOD/LtJ, NON/LtJ,

162 In a yeast expression system, ALR-1 enhances MEC-3/UNC-86-dependent transcription from

163 In all in vivo systems, ALR and F1 female recipients of NOD marrow remained IDDM

164 tend our previously advanced hypothesis that ALR and other hepatotrophic factors play an important ro

165 Our data imply that ALR is critical for cell survival under nutrient stress

166 Our studies indicate that ALR-1 is required for maintenance of the amphid organ st

167 iption from the mec-3 promoter, showing that ALR-1 can enhance bulk mec-3 expression.

168 Genetic interaction tests also suggest that ALR-1 may function cooperatively with the cell adhesion

169 oblasts with GBA1 mutations, suggesting that ALR is compromised.

170 We found that ALRs are dispensable for the type I interferon (IFN) res

171 We also found that ALRs do not contribute to autoimmune disease in the Trex

172 Our findings indicate that ALRs are dispensable for the ISD response and suggest th

173 The ALR gene and gene product were subsequently described, b

174 The ALR gene was mapped to chromosome band 12q12-13, adjacen

175 The ALR is unlikely to contribute to horizontal strabismus i

176 ds to this element but does not assemble the ALR complex.

177 Contrary to previous belief, the ALR peptide and ALR mRNA were present in comparable conc

178 In conclusion, the ALR mouse presents a unique opportunity to identify domi

179 In conclusion, the ALR/Lt mouse may provide important insight into genetic

180 As expected, the ALR active site was enlarged by the Y274F substitution,

181 proximately 57% and approximately 84% in the ALR and BCR, respectively.

182 ALR, characterizing at the atomic level the ALR intermediates that allow electrons to rapidly flow t

183 PTIP promotes assembly of the ALR complex and H3K4 methylation at a PAX2-binding DNA e

184 Characteristics of the ALR did not correlate with strabismus.

185 hat Mnr2 could function independently of the ALR genes.

186 uncovered 16 positives, one of which was the ALR gene, whose similarity to three of gliadin's five do

187 Whether the ALRs are essential for activation of this pathway remain

188 This ALR/Lt islet resistance extended to hydrogen peroxide, a

189 Thus, ALR appears to be constitutively expressed in hepatocyte

190 , which competes with ASC for recruitment to ALRs, as an inhibitor of DNA virus-induced activation of

191 This inability of cytokine-treated ALR islets to up-regulate inducible NO synthase and prod

192 These findings validate ALRs as key activators of the antiviral response and pro

193 rror-prone PCR and selection yielded variant ALR(Y274F), which catalyzes cystathionine beta-eliminati

194 d hyperglycemia and hypoinsulinemia, whereas ALR/Lt mice maintained normal plasma insulin and glucose

195 Ag7 identical) allowed us to examine whether ALR islet cells could survive autoimmune destruction by

196 cyte was the predominant liver cell in which ALR was synthesized and stored; the cultured hepatocytes

197 ve phosphorylation and in cell growth, while ALR has hepatotrophic activity.

198 tyrosine formation may, in part, explain why ALR mice are resistant to type 1 diabetes when reconstit

199 Consistent with ALR-1 expression within the amphid socket cell, our resu

200 PTIP copurifies with ALR, MLL3, and other components of a histone methyltrans

201 astic mouse strains were generated: one with ALR nuclear DNA and NOD mtDNA (ALR.mt(NOD)) and the reci

202 Of the group treated with ALR at 40 ng/kg/day for 14 days, 89% (eight of nine) wer

203 Of the group treated with ALR at 400 ng/kg/day for 14 days, 88% (seven of eight) w

204 re the mechanism of the electron flux within ALR, characterizing at the atomic level the ALR intermed