iiinova — where your digital self keeps evolving

Reposted by Raffaella De Pace

Juan S. Bonifacino @juanbonifacino.bsky.social · 23/07/2026

New study from our lab at NICHD, NIH! We identify TBC1D9 and TBC1D9B as novel ARL8 effectors that suppress exosome secretion by inactivating RAB11A and thus blocking exocyst-dependent fusion of multivesicular endosomes with the plasma membrane. www.pnas.org/doi/10.1073/...

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Juan S. Bonifacino @juanbonifacino.bsky.social · 25/04/2026

Many thanks, @depaceraffa.bsky.social! I am deeply honored by this recognition and grateful to all who made it possible. 🙏

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Raffaella De Pace @depaceraffa.bsky.social · 25/04/2026

Congratulations to @juanbonifacino.bsky.social at the NIH on his election to the American Academy of Arts and Sciences — one of the nation's most prestigious honorary societies — in recognition of his distinguished contributions to cellular and developmental biology! 🎉 www.amacad.org/new-members-...

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Juan S. Bonifacino @juanbonifacino.bsky.social · 24/04/2026

New work from Niccolo Mencacci @feinberg.northwestern.edu et al., with contribution from @depaceraffa.bsky.social in our lab at the NIH, identifies pathogenic variants in BORCS5, encoding a subunit of the BORC complex, as a cause of a severe neurodevelopmental disorder. www.jci.org/articles/vie...

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Raffaella De Pace @depaceraffa.bsky.social · 26/03/2026

Take a look at our Research Highlight: Adriana Golding et al. www.nature.com/articles/s41... We highlight the work by Ghoochani et al., who generated an atlas of brain lysosomal proteins and identified SLC45A1-associated disease as a lysosomal disorder: www.cell.com/cell/fulltex...

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Raffaella De Pace @depaceraffa.bsky.social · 26/03/2026

🔥Excited to share our new paper! We describe patients from seven independent families carrying pathogenic BLOC1S1 variants, presenting with severe neurodevelopmental disorder. These variants disrupt BLOC-1/BORC complexes. Congrats to all co-authors! 👏 authors.elsevier.com/c/1mqGAgeXHm-E

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Juan S. Bonifacino @juanbonifacino.bsky.social · 23/12/2025

Latest preprint from our lab reports that the distinct pH of anterograde (less acidic) and retrograde (more acidic) lysosomal vesicles in the axon depends on assembly of the V1 and V0 domains of the vacuolar H+ ATPase, mediated by the metazoan RAVE complex www.biorxiv.org/content/10.6...

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Pedro Rocha @pedroprocha.bsky.social · 08/09/2025

🚨🚨🚨 Please repost We are looking for postdocs to join our lab at NIH. Apply: www.nichd.nih.gov/research/atNICHD/Investigators/rocha/apply Learn more about training at NIH : www.training.nih.gov/research-tra...

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Cell Chemical Biology @cp-cellchembiol.bsky.social · 19/09/2025

BLOC-1 and BORC: Complex regulators of endolysosomal dynamics dlvr.it/TN9mpn

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Juan S. Bonifacino @juanbonifacino.bsky.social · 21/09/2025

Thank you, @mishtudey.bsky.social and @cp-cellchembiol.bsky.social‬ for featuring this interview with @depaceraffa.bsky.social and Chad Williamson in connection to our article on BLOC-1 and BORC dlvr.it/TN9mpn

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Carlos Guardia @charlyguardia.bsky.social · 16/04/2025

Job ad is here! Check it out :)

training.nih.gov

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Juan S. Bonifacino @juanbonifacino.bsky.social · 21/08/2025

👉 New publication ahead of print from our lab: tinyurl.com/ye7pntte We show that the protein SPG21, mutated in hereditary spastic paraplegia 21, localizes to endolysosomes via RAB7A, where it promotes mTORC1-dependent TFEB phosphorylation, reducing expression of a subset of TFEB regulated genes

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Juan S. Bonifacino @juanbonifacino.bsky.social · 24/08/2025

New from @depaceraffa.bsky.social from our lab #NICHD #NIH in collaboration with Adeline Vanderver @childrensphila.bsky.social ‬and colleagues reporting mutations in the BLOC1S1 subunit of the BLOC-1 and BORC complexes in children with a neurodevelopmental disorder www.medrxiv.org/content/10.1...

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Juan S. Bonifacino @juanbonifacino.bsky.social · 25/08/2025

Happy to share an exciting study from Yihong Ye’s lab at NIH, with a minor contribution from our lab: ceroid lipofuscinosis-4 (CLN4)-linked DNAJC5 mutations cause lysosomal damage as a driver of neurodegeneration in iPSC-derived neurons. CHIP safeguards lysosomes via microautophagy 👉 rdcu.be/eChof

rdcu.be

CHIP protects lysosomes from CLN4 mutant-induced membrane damage

Nature Cell Biology - Lee et al. use an aggregation-prone CLN4 mutant that causes lysosomal damage in neurons and show that in non-neurons, the ubiquitin ligase CHIP prevents CLN4-dependent...

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Juan S. Bonifacino @juanbonifacino.bsky.social · 26/08/2025

Excited to share our new review with @depaceraffa.bsky.social‬, @saikat2025.bsky.social‬, and Chad Williamson on the BLOC-1 and BORC complexes—key regulators of endolysosomal processes and linked to several genetic diseases. #NIH #Lysosomes #RareDiseases authors.elsevier.com/a/1lg4i8jWWJ...

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Raffaella De Pace @depaceraffa.bsky.social · 14/04/2025

🚨 Exciting postdoc opportunity in @juanbonifacino.bsky.social lab!! If you have a passion for protein trafficking and neurodevelopmental disorders, and want to join a dynamic and collaborative team consider applying! Positions will be available starting in October.🍁🍂

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Prasanna Satpute-Krishnan @pskcellbio.bsky.social · 05/12/2024

I’m so excited to organize the #Lysosomes Subgroup at @ASCBiology 2024 meeting on Sunday, Dec. 15 at 3:15pm, Room 33B. Lysosomes naturally lend themselves to puns. So…Don’t be 𝒃𝒂𝒔𝒊𝒄. Join us for fresh, 𝒄𝒖𝒕𝒕𝒊𝒏𝒈-edge talks and puntastic times. It will 𝒆𝒂𝒕 𝒂𝒕 𝒚𝒐𝒖 if you miss it.

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Juan S. Bonifacino @juanbonifacino.bsky.social · 19/11/2024

This post is about a commentary that I wrote with Xin Yong about recognition of cargo vesicles by tethering factors involved in endosomes to TGN retrograde transport.

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Juan S. Bonifacino @juanbonifacino.bsky.social · 23/11/2024

Kudos to authors Morié Ishida, Adriana Golding, @TalKerenKaplan @NICHD_NIH and our collaborators Tamas Balla @NICHD_NIH and Yan Li @NIH_NINDS! Great teamwork!

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Juan S. Bonifacino @juanbonifacino.bsky.social · 23/11/2024

Excited to share our latest findings on how the phosphoinositide PI4P pool at the trans-Golgi network (TGN) is regulated through a SYS1-ARFRP1-ARL5-ARMH3-PI4KB pathway, in which ARMH3 (also known as C10orf76) acts as an ARL5 effector to activate PI4KB. rdcu.be/d1dZ2

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Juan S. Bonifacino @juanbonifacino.bsky.social · 23/11/2024

Reposting @yousufakhan.bsky.social reference to a preprint demonstrating that programmed ribosomal frameshifting generates a PLEKHM2 proteoform that behaves as a constitutively active adaptor for ARL8-dependent coupling of lysosomes to kinesin-1, with collaboration from our lab tinyurl.com/mtrkzdrm

tinyurl.com

Programmed ribosomal frameshifting during PLEKHM2 mRNA decoding generates a constitutively active mediator of kinesin-1-dependent lysosome transport

Programmed ribosomal frameshifting is a translational recoding phenomenon in which a proportion of ribosomes are stimulated to slip backwards or forwards on an mRNA1, rephasing the ribosome relative t...

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