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1 sis of lysosome-related organelle complexes (BLOCs).
2 is of lysosome-related organelles complexes (BLOCs).
3 idneys should be used as single grafts or en bloc.
4 2 locus and the capture of the cps island en bloc.
5 uncovered novel allele-specific features of BLOCs.
9 We discuss similarities and differences of BLOC-1 activity in the biogenesis of gut granules as com
15 novel vesicle transport mechanism requiring BLOC-1 and AP-3 complexes for cargo sorting from neurona
19 eam endosomal intermediates, suggesting that BLOC-1 and BLOC-2 act sequentially in the same pathway.
20 quent observations that AP-3, as well as the BLOC-1 and BLOC-2 Hermansky-Pudlak syndrome proteins are
21 mediated by AP-3 and one pathway mediated by BLOC-1 and BLOC-2, that are deficient in several forms o
23 Dennis et al. report differential roles for BLOC-1 and BLOC-3 complexes in delivery and recycling of
24 hypopigmentation phenotypes associated with BLOC-1 and BLOC-3 deficiency in Hermansky-Pudlak syndrom
25 e that the Hermansky-Pudlak syndrome complex BLOC-1 and its cargo PI4KIIalpha interact with regulator
27 omal sorting mechanisms mediated by AP-3 and BLOC-1 are perturbed in Hermansky-Pudlak Syndrome, a hum
28 ient mice suggesting that genetic defects in BLOC-1 are upstream of the BDNF phenotype in Mecp2 defic
30 2 transport to melanosomes and indicate that BLOC-1 can cooperate with either adaptor during cargo so
31 dies expand the functional repertoire of the BLOC-1 complex and provide insight into putative molecul
33 that Arp2/3, dysbindin, and subunits of the BLOC-1 complex biochemically and genetically interact, m
35 ed prevalent and novel cellular roles of the BLOC-1 complex in neuronal cells by performing large-sca
36 subunits, some of which are shared with the BLOC-1 complex involved in the biogenesis of lysosome-re
40 and its product dysbindin, a subunit of the BLOC-1 complex, and describe a neuronal pathway modulati
41 entified 24 proteins that associate with the BLOC-1 complex, many of which were altered in content/di
45 SNAREs; subunits of the AP-3, retromer, and BLOC-1 complexes; lysosomal enzymes; CHC22; and five nov
52 hinery as factors downregulated in dysbindin/BLOC-1 deficiency in neuroectodermal cells and iPSC-deri
53 ts into the metabolic consequences caused by BLOC-1 deficiency in pallid mice, which carry a null mut
56 NF content was reduced in the hippocampus of BLOC-1 deficient mice suggesting that genetic defects in
61 ogaster as a powerful model for the study of BLOC-1 function and its interactions with modifier genes
64 as compared to mammalian melanosomes, where BLOC-1 has been most extensively studied for its role in
65 -3(-) and BLOC-1(-) phenotypes revealed that BLOC-1 has some functions independent of the AP-3 adapto
66 his is the first demonstration of a role for BLOC-1 in ciliary assembly and highlights the complexity
73 of the Arp2/3 complex were downregulated by BLOC-1 loss of function, thus affecting actin dynamics i
79 elocalization is prevented by loss of either BLOC-1 or Msb3, but it also occurs in mutants lacking en
80 o test the hypothesis that NSF and dysbindin/BLOC-1 participate in a pathway-regulating synaptic func
83 y with an increase in the expression of both BLOC-1 related complex subunit 7 (BORCS7) and a previous
84 ks, or interactomes, downstream of dysbindin/BLOC-1 remain partially explored despite their potential
86 nterneuron markers, as well as expression of BLOC-1 subunit gene products, were affected differently
87 n glo-2 and snpn-1,or RNAi targeting 5 other BLOC-1 subunit homologues in a genetic background sensit
96 In metazoan cells, three complexes, termed BLOC-1 to -3, mediate protein sorting from the early end
97 egulate dysbindin, compromise the ability of BLOC-1 to traffic DRD2 toward degradation, but has littl
99 utant analysis and comparison of AP-3(-) and BLOC-1(-) phenotypes revealed that BLOC-1 has some funct
101 is of Lysosome-Related Organelles Complex 1 (BLOC-1) is a protein complex containing the schizophreni
102 is of lysosome-related organelles complex 1 (BLOC-1) is a protein complex formed by the products of e
103 is of lysosome-related organelles complex-1 (BLOC-1) is a protein complex involved in the formation o
104 is of lysosome-related organelles complex 1 (BLOC-1) required for lysosome-related organelle biogenes
106 is of lysosome-related organelles complex 1 (BLOC-1), increased the content of characteristic synapti
107 is of lysosome-related organelles complex-1 (BLOC-1), interact with and are regulated by the lipid ki
108 is of lysosome-related organelles complex 1 (BLOC-1), which interacts with the adaptor protein comple
115 udlak syndrome complexes, we identified that BLOC-1, but not BLOC-2 or BLOC-3, deficiencies affect PI
116 , and down-regulation of either PI4KIIalpha, BLOC-1, or AP-3 complexes led to similar LAMP1 phenotype
121 assemble into five distinct complexes (AP-3, BLOC-1-3, and HOPS) that either sort membrane proteins o
127 n, we examined the role for NSF in dysbindin/BLOC-1-dependent synaptic homeostatic plasticity in Dros
128 r data suggest that VAMP7 mediates fusion of BLOC-1-dependent transport carriers with melanosomes, il
129 les, our data reveal that dysfunction of the BLOC-1-KIF13A-Annexin A2 molecular network underlies the
132 show that Ragulator directly interacts with BLOC-1-related complex (BORC), a multi-subunit complex p
133 C-terminal domain of lyspersin, a subunit of BLOC-1-related complex (BORC), is essential and sufficie
135 ntent and/or subcellular distribution of the BLOC-1-sensitive cargoes PI4KIIalpha, ATP7A, and VAMP7.
142 ng: LE transport regulates SV pool size, and BLOC-1/AP-3-dependent sorting fine-tunes the Ca(2+) sens
144 nderwent transthoracic esophagectomy with en bloc 2-field lymphadenectomy after neoadjuvant therapy w
145 le after transthoracic esophagectomy with en bloc 2-field lymphadenectomy in patients post neoadjuvan
148 ranule biogenesis requires the activities of BLOC-2 and a putative Rab guanine-nucleotide-exchange fa
151 vations that AP-3, as well as the BLOC-1 and BLOC-2 Hermansky-Pudlak syndrome proteins are essential
153 omplexes, we identified that BLOC-1, but not BLOC-2 or BLOC-3, deficiencies affect PI4KIIalpha inclus
155 t melanocytes from mouse HPS models to place BLOC-2 within a cargo transport pathway from recycling e
156 sis of lysosome-related organelle complex-2 (BLOC-2) (HPS-5), BLOC-3 (HPS-1), and adaptin-3 (HPS-2).
158 ubtypes result from mutations in subunits of BLOC-2, a protein complex with no known molecular functi
159 el mice lacking a different protein complex, BLOC-2, accumulate Tyrp1 in distinct downstream endosoma
162 steady state levels of known cargoes of the BLOC-2, AP-3, and AP-1 pathways, the melanin-synthesizin
166 uring melanosomes; in contrast, tubules from BLOC-2-deficient cells were shorter in length and made f
168 eys not recovered, 21 not transplanted, 8 en bloc, 23 with extrarenal organs, and 6 with missing reco
169 e gallbladder and extrahepatic bile ducts en bloc 3, 7, and 14 days later, generated biotinylated RNA
171 esis of lysosome-related organelles complex (BLOC)-3, and patients with BLOC-3 or AP-3 deficiency dev
173 ing Rab-32 or its nucleotide exchange factor BLOC-3 are permissive to S. Typhi infection and exhibit
176 earlier work, we found that a subunit of the BLOC-3 complex inhibits loading of Argonaute (Ago) prote
177 al. report differential roles for BLOC-1 and BLOC-3 complexes in delivery and recycling of melanosoma
178 tation phenotypes associated with BLOC-1 and BLOC-3 deficiency in Hermansky-Pudlak syndrome variants.
179 Our results imply that (1) AP-3, BLOC-1, and BLOC-3 facilitate protein sorting to lysosomes to suppor
185 ganelles complex (BLOC)-3, and patients with BLOC-3 or AP-3 deficiency develop pulmonary fibrosis.
189 reveals a specific and strong interaction of BLOC-3 with the GTP-bound form of the endosomal GTPase,
192 we identified that BLOC-1, but not BLOC-2 or BLOC-3, deficiencies affect PI4KIIalpha inclusion into A
195 ARE recycling from melanosomes as a critical BLOC-3-dependent step, and likely explain the distinct h
199 review was performed on all 72 pediatric en bloc and 75 live adult donor kidney recipients transplan
201 tient survival rates were similar between en bloc and live donor groups (97.3% vs. 98.6%, P = 0.585 a
205 ace forms before Ca(2+) triggering, moves en bloc as Ca(2+) influx promotes the interactions between
206 two monkey orbits were serially sectioned en bloc at 10-mum thickness in the coronal plane and staine
209 rench and by eventual alignment with eastern bloc Communist countries, thus isolating much of his wor
210 n of Rab32 or of an essential component of a BLOC complex was sufficient to allow S. Typhi to survive
214 paring esophagectomy with transhiatal and en bloc esophagectomy in patients with intramucosal adenoca
216 All patients had undergone transthoracic en bloc esophagectomy, with a median of 27 resected lymph n
223 ordomas of the mobile spine and sacrum is en-bloc excision with wide margins and postoperative extern
224 three patients were surgically extracted en bloc for histologic and microcomputed tomography (micro-
226 n of Poh1, which removes ubiquitin chains en bloc from proteasomal substrates prior to their degradat
228 was detected later posttransplantation in en bloc group (45.6 +/- 33.6 months vs. 23.4 +/- 16.3 month
230 satisfactory when transplanted as SKs or en bloc; however, the absence of an aortic patch in SK tran
231 genomes are transmitted from cell-to-cell en bloc in membrane-bound PS vesicles instead of as single
233 Whole orbits were then serially sectioned en bloc in the quasi-coronal plane, stained for connective
235 e segment from chromosome 15 was inserted en bloc into the second intron of the RARA gene on chromoso
236 whereas broad local enrichment of H3K27me3 (BLOC) is a domain-wide feature at imprinted clusters.
237 tudy is to compare graft survival (GS) of en bloc kidney (EBK) from young pediatric donors to other d
241 c single kidney transplant (SKT, n=3712), en bloc kidney transplant (EBK, n=1517), or adult standard
242 cellent long-term outcome after pediatric en bloc kidney transplantation from donors weighing less th
245 there has been hesitancy in transplanting en bloc kidneys from donors weighing less than 10 kg due to
247 elp stratify patients receiving pediatric en bloc kidneys into risk categories for vascular thrombosi
249 leviate burden on waiting list, pediatric en bloc kidneys should be transplanted more often when avai
251 thogenicity locus presumably acquired via en bloc mobilization from a direct predecessor of equine pV
252 y-associating domains (TADs) that undergo en bloc movements, and identify dynamically coupled distal
253 ive morbidity and mortality rates, and an en bloc multivisceral resection should be performed in pati
254 ed seventy-one patients had transthoracic en bloc (n = 161) or transhiatal esophagectomy (n = 10) for
255 ormed to compare outcomes after pediatric en bloc (n=20, mean donor weight 11.4 kg), standard criteri
257 weight, 27 kg); SCDKT (n=283); pediatric en bloc (n=21), living-donor (n=275), and extended criteria
261 graft losses can be minimized when using en bloc pediatric kidneys from donors weighing less than or
262 lthough pancreaticoduodenectomy (PD) with en-bloc portal vein/superior mesenteric vein (PV/SMV) resec
263 rhythmic inspiratory activity in isolated en bloc preparations even after acidic or serotonergic stim
264 llowed to extend membrane protrusions in the BloC-Printing device for 3 h, multiple biophysical chara
267 ing technique, termed "Block-Cell-Printing" (BloC-Printing), allows for convenient, precise, multiple
271 h albendazole was instituted and surgical en bloc removal of the cyst was obtained, allowing the pati
272 ombosis remain a challenge with pediatric en bloc renal transplants, but once the allografts survive
273 lar polysaccharides has proceeded through en bloc replacement of individual glycosyltransferase genes
274 cosal resection was developed to increase en-bloc resection (less residual disease) of a flat colorec
282 al time, but is promising due to the high en-bloc resection rates and the very low recurrence rates.
285 Nine months after surgical correction, en bloc resections were obtained and examined histologicall
287 To avoid this problem, we tested several en bloc staining techniques to contrast tissue in serial se
288 this step with other classic heavy metal en bloc stains, including uranyl acetate (UA), lead asparta
290 to a polyisoprenyl donor, followed by the en bloc transfer of the glycan to particular asparagine res
291 n undecaprenyl diphosphate and subsequent en bloc transfer of the glycan to serine residues of select
292 rase (OST), the enzyme that catalyzes the en bloc transfer of the oligosaccharide onto the acceptor a
300 ed that the left-handed dimer gets tilted en bloc, whereas conformational transitions to alternative
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