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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.
7 We discuss similarities and differences of BLOC-1 activity in the biogenesis of gut granules as com
10 novel vesicle transport mechanism requiring BLOC-1 and AP-3 complexes for cargo sorting from neurona
13 quent observations that AP-3, as well as the BLOC-1 and BLOC-2 Hermansky-Pudlak syndrome proteins are
15 Dennis et al. report differential roles for BLOC-1 and BLOC-3 complexes in delivery and recycling of
16 hypopigmentation phenotypes associated with BLOC-1 and BLOC-3 deficiency in Hermansky-Pudlak syndrom
17 e that the Hermansky-Pudlak syndrome complex BLOC-1 and its cargo PI4KIIalpha interact with regulator
19 omal sorting mechanisms mediated by AP-3 and BLOC-1 are perturbed in Hermansky-Pudlak Syndrome, a hum
20 ient mice suggesting that genetic defects in BLOC-1 are upstream of the BDNF phenotype in Mecp2 defic
22 2 transport to melanosomes and indicate that BLOC-1 can cooperate with either adaptor during cargo so
23 dies expand the functional repertoire of the BLOC-1 complex and provide insight into putative molecul
25 that Arp2/3, dysbindin, and subunits of the BLOC-1 complex biochemically and genetically interact, m
26 ed prevalent and novel cellular roles of the BLOC-1 complex in neuronal cells by performing large-sca
27 subunits, some of which are shared with the BLOC-1 complex involved in the biogenesis of lysosome-re
30 and its product dysbindin, a subunit of the BLOC-1 complex, and describe a neuronal pathway modulati
31 entified 24 proteins that associate with the BLOC-1 complex, many of which were altered in content/di
41 hinery as factors downregulated in dysbindin/BLOC-1 deficiency in neuroectodermal cells and iPSC-deri
42 ts into the metabolic consequences caused by BLOC-1 deficiency in pallid mice, which carry a null mut
44 NF content was reduced in the hippocampus of BLOC-1 deficient mice suggesting that genetic defects in
49 ogaster as a powerful model for the study of BLOC-1 function and its interactions with modifier genes
51 as compared to mammalian melanosomes, where BLOC-1 has been most extensively studied for its role in
52 -3(-) and BLOC-1(-) phenotypes revealed that BLOC-1 has some functions independent of the AP-3 adapto
53 his is the first demonstration of a role for BLOC-1 in ciliary assembly and highlights the complexity
59 of the Arp2/3 complex were downregulated by BLOC-1 loss of function, thus affecting actin dynamics i
64 elocalization is prevented by loss of either BLOC-1 or Msb3, but it also occurs in mutants lacking en
65 o test the hypothesis that NSF and dysbindin/BLOC-1 participate in a pathway-regulating synaptic func
68 y with an increase in the expression of both BLOC-1 related complex subunit 7 (BORCS7) and a previous
69 ks, or interactomes, downstream of dysbindin/BLOC-1 remain partially explored despite their potential
71 nterneuron markers, as well as expression of BLOC-1 subunit gene products, were affected differently
72 n glo-2 and snpn-1,or RNAi targeting 5 other BLOC-1 subunit homologues in a genetic background sensit
80 In metazoan cells, three complexes, termed BLOC-1 to -3, mediate protein sorting from the early end
82 utant analysis and comparison of AP-3(-) and BLOC-1(-) phenotypes revealed that BLOC-1 has some funct
84 is of Lysosome-Related Organelles Complex 1 (BLOC-1) is a protein complex containing the schizophreni
85 is of lysosome-related organelles complex 1 (BLOC-1) is a protein complex formed by the products of e
86 is of lysosome-related organelles complex-1 (BLOC-1) is a protein complex involved in the formation o
87 is of lysosome-related organelles complex 1 (BLOC-1) required for lysosome-related organelle biogenes
89 is of lysosome-related organelles complex 1 (BLOC-1), increased the content of characteristic synapti
90 is of lysosome-related organelles complex-1 (BLOC-1), interact with and are regulated by the lipid ki
91 is of lysosome-related organelles complex 1 (BLOC-1), which interacts with the adaptor protein comple
97 udlak syndrome complexes, we identified that BLOC-1, but not BLOC-2 or BLOC-3, deficiencies affect PI
98 , and down-regulation of either PI4KIIalpha, BLOC-1, or AP-3 complexes led to similar LAMP1 phenotype
106 n, we examined the role for NSF in dysbindin/BLOC-1-dependent synaptic homeostatic plasticity in Dros
107 r data suggest that VAMP7 mediates fusion of BLOC-1-dependent transport carriers with melanosomes, il
108 les, our data reveal that dysfunction of the BLOC-1-KIF13A-Annexin A2 molecular network underlies the
111 show that Ragulator directly interacts with BLOC-1-related complex (BORC), a multi-subunit complex p
112 C-terminal domain of lyspersin, a subunit of BLOC-1-related complex (BORC), is essential and sufficie
114 ntent and/or subcellular distribution of the BLOC-1-sensitive cargoes PI4KIIalpha, ATP7A, and VAMP7.
121 ng: LE transport regulates SV pool size, and BLOC-1/AP-3-dependent sorting fine-tunes the Ca(2+) sens
123 nderwent transthoracic esophagectomy with en bloc 2-field lymphadenectomy after neoadjuvant therapy w
124 le after transthoracic esophagectomy with en bloc 2-field lymphadenectomy in patients post neoadjuvan
126 ranule biogenesis requires the activities of BLOC-2 and a putative Rab guanine-nucleotide-exchange fa
133 vations that AP-3, as well as the BLOC-1 and BLOC-2 Hermansky-Pudlak syndrome proteins are essential
136 omplexes, we identified that BLOC-1, but not BLOC-2 or BLOC-3, deficiencies affect PI4KIIalpha inclus
137 ore, releasates of immature WPBs from either BLOC-2 or exocyst-depleted endothelial cells lacked high
138 t melanocytes from mouse HPS models to place BLOC-2 within a cargo transport pathway from recycling e
139 sis of lysosome-related organelle complex-2 (BLOC-2) (HPS-5), BLOC-3 (HPS-1), and adaptin-3 (HPS-2).
140 biogenesis of lysosomal related organelle-2 (BLOC-2) functions in the biogenesis of platelet dense gr
141 ubtypes result from mutations in subunits of BLOC-2, a protein complex with no known molecular functi
144 steady state levels of known cargoes of the BLOC-2, AP-3, and AP-1 pathways, the melanin-synthesizin
147 uring melanosomes; in contrast, tubules from BLOC-2-deficient cells were shorter in length and made f
149 ome-related organelles, we hypothesized that BLOC-2-dependent endolysosomal trafficking is essential
151 orms of VWF, demonstrating the importance of BLOC-2/exocyst-mediated endosomal input during VWF matur
152 eys not recovered, 21 not transplanted, 8 en bloc, 23 with extrarenal organs, and 6 with missing reco
154 esis of lysosome-related organelles complex (BLOC)-3, and patients with BLOC-3 or AP-3 deficiency dev
156 ing Rab-32 or its nucleotide exchange factor BLOC-3 are permissive to S. Typhi infection and exhibit
159 earlier work, we found that a subunit of the BLOC-3 complex inhibits loading of Argonaute (Ago) prote
160 al. report differential roles for BLOC-1 and BLOC-3 complexes in delivery and recycling of melanosoma
161 tation phenotypes associated with BLOC-1 and BLOC-3 deficiency in Hermansky-Pudlak syndrome variants.
162 Our results imply that (1) AP-3, BLOC-1, and BLOC-3 facilitate protein sorting to lysosomes to suppor
168 ganelles complex (BLOC)-3, and patients with BLOC-3 or AP-3 deficiency develop pulmonary fibrosis.
172 reveals a specific and strong interaction of BLOC-3 with the GTP-bound form of the endosomal GTPase,
175 we identified that BLOC-1, but not BLOC-2 or BLOC-3, deficiencies affect PI4KIIalpha inclusion into A
178 ARE recycling from melanosomes as a critical BLOC-3-dependent step, and likely explain the distinct h
186 ace forms before Ca(2+) triggering, moves en bloc as Ca(2+) influx promotes the interactions between
187 y (DBS)) is placed over RTN in an ex-vivo en bloc brain and changes in cation concentration in the di
189 rench and by eventual alignment with eastern bloc Communist countries, thus isolating much of his wor
190 mor extirpation via either en-bloc or non-en-bloc complete resection based on FS analysis is associat
191 n of Rab32 or of an essential component of a BLOC complex was sufficient to allow S. Typhi to survive
193 oss 3 groups: complete resection achieved en-bloc (CR-EB), complete resection achieved non-en-bloc (C
195 scores to match 148 en bloc with 581 non-en bloc deceased donor recipients (matching variables: tran
197 All patients had undergone transthoracic en bloc esophagectomy, with a median of 27 resected lymph n
202 ordomas of the mobile spine and sacrum is en-bloc excision with wide margins and postoperative extern
203 three patients were surgically extracted en bloc for histologic and microcomputed tomography (micro-
205 n of Poh1, which removes ubiquitin chains en bloc from proteasomal substrates prior to their degradat
208 a case series of adult patients receiving en-bloc heart-liver transplantation (HLTx), describe techni
209 etrospective review of patients receiving en-bloc HLTx over 18 months, with clinical follow up to 1 y
211 satisfactory when transplanted as SKs or en bloc; however, the absence of an aortic patch in SK tran
212 genomes are transmitted from cell-to-cell en bloc in membrane-bound PS vesicles instead of as single
215 e segment from chromosome 15 was inserted en bloc into the second intron of the RARA gene on chromoso
216 whereas broad local enrichment of H3K27me3 (BLOC) is a domain-wide feature at imprinted clusters.
217 ient and death-censored graft survival of en bloc kidney (EBK) and split kidney (SpK) transplants fro
218 tudy is to compare graft survival (GS) of en bloc kidney (EBK) from young pediatric donors to other d
222 c single kidney transplant (SKT, n=3712), en bloc kidney transplant (EBK, n=1517), or adult standard
223 cellent long-term outcome after pediatric en bloc kidney transplantation from donors weighing less th
226 there has been hesitancy in transplanting en bloc kidneys from donors weighing less than 10 kg due to
228 elp stratify patients receiving pediatric en bloc kidneys into risk categories for vascular thrombosi
230 thogenicity locus presumably acquired via en bloc mobilization from a direct predecessor of equine pV
231 y-associating domains (TADs) that undergo en bloc movements, and identify dynamically coupled distal
232 ive morbidity and mortality rates, and an en bloc multivisceral resection should be performed in pati
233 ed seventy-one patients had transthoracic en bloc (n = 161) or transhiatal esophagectomy (n = 10) for
234 ormed to compare outcomes after pediatric en bloc (n=20, mean donor weight 11.4 kg), standard criteri
235 weight, 27 kg); SCDKT (n=283); pediatric en bloc (n=21), living-donor (n=275), and extended criteria
236 my, complete tumor extirpation via either en-bloc or non-en-bloc complete resection based on FS analy
241 graft losses can be minimized when using en bloc pediatric kidneys from donors weighing less than or
242 lthough pancreaticoduodenectomy (PD) with en-bloc portal vein/superior mesenteric vein (PV/SMV) resec
243 rhythmic inspiratory activity in isolated en bloc preparations even after acidic or serotonergic stim
244 llowed to extend membrane protrusions in the BloC-Printing device for 3 h, multiple biophysical chara
247 ing technique, termed "Block-Cell-Printing" (BloC-Printing), allows for convenient, precise, multiple
250 Compared with non-en bloc recipients, en bloc recipients had lower 1-y graft survival (78.9% vers
253 lant Recipients to identify 149 pediatric en bloc recipients transplanted from October 1, 1987 to Dec
256 h albendazole was instituted and surgical en bloc removal of the cyst was obtained, allowing the pati
257 cosal resection was developed to increase en-bloc resection (less residual disease) of a flat colorec
260 22.9%; PTx and hemithyroidectomy: 24.1%; en bloc resection 15.7%; others 37.3%] and complications of
264 eness and safety of TAMIS and ESD for the en bloc resection of large non-pedunculated rectal lesions.
266 went extended radical pelvic surgery with en bloc resection of the sciatic or femoral nerves at a sin
267 al time, but is promising due to the high en-bloc resection rates and the very low recurrence rates.
269 e become available for minimally invasive en bloc resections of large non-pedunculated rectal lesions
270 Nine months after surgical correction, en bloc resections were obtained and examined histologicall
276 To avoid this problem, we tested several en bloc staining techniques to contrast tissue in serial se
277 ively to UA in negative staining (NS), in en bloc staining, and post sectioning staining (PSS) of bio
278 this step with other classic heavy metal en bloc stains, including uranyl acetate (UA), lead asparta
281 to a polyisoprenyl donor, followed by the en bloc transfer of the glycan to particular asparagine res
282 n undecaprenyl diphosphate and subsequent en bloc transfer of the glycan to serine residues of select
283 rase (OST), the enzyme that catalyzes the en bloc transfer of the oligosaccharide onto the acceptor a
290 ank test and examined survival benefit of en bloc transplantation versus remaining on the waiting lis
295 es in general, transport multiple virions en bloc via infectious extracellular vesicles, 100~1000 nm
299 ed that the left-handed dimer gets tilted en bloc, whereas conformational transitions to alternative
300 We used propensity scores to match 148 en bloc with 581 non-en bloc deceased donor recipients (mat