Proteomics

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Neurochondrin interacts with the SMN protein suggesting a novel mechanism for Spinal Muscular Atrophy pathology.


ABSTRACT: Spinal Muscular Atrophy (SMA) is an inherited neurodegenerative condition caused by reduction in functional Survival Motor Neurones Protein (SMN). SMN has been implicated in transport of mRNA in neural cells for local translation. We previously identified microtubule-dependant mobile vesicles rich in SMN and SmB, a member of the Sm family of snRNP-associated proteins, in neural cells. By comparing the interactomes of SmB and SmN, a neural-specific Sm protein, we now show that the essential neural protein neurochondrin (NCDN) interacts with Sm proteins and SMN in the context of mobile vesicles in neurites. NCDN has roles in protein localisation in neural cells, and in maintenance of cell polarity. NCDN is required for the correct localisation of SMN, suggesting they may both be required for formation and transport of trafficking vesicles. NCDN may have potential as a therapeutic target for SMA together with, or in place of, those targeting SMN expression

INSTRUMENT(S): TripleTOF 5600

ORGANISM(S): Homo Sapiens (human)

SUBMITTER: Sally Shirran  

LAB HEAD: Judith Elizabeth Sleeman

PROVIDER: PXD008710 | Pride | 2018-03-15

REPOSITORIES: Pride

Dataset's files

Source:
Action DRS
LukeBB2ul.wiff Wiff
LukeBB2ul.wiff.mtd Wiff
LukeBB2ul.wiff.scan Wiff
LukeBM2ul.wiff Wiff
LukeBM2ul.wiff.mtd Wiff
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Neurochondrin interacts with the SMN protein suggesting a novel mechanism for spinal muscular atrophy pathology.

Thompson Luke W LW   Morrison Kim D KD   Shirran Sally L SL   Groen Ewout J N EJN   Gillingwater Thomas H TH   Botting Catherine H CH   Sleeman Judith E JE  

Journal of cell science 20180417 8


Spinal muscular atrophy (SMA) is an inherited neurodegenerative condition caused by a reduction in the amount of functional survival motor neuron (SMN) protein. SMN has been implicated in transport of mRNA in neural cells for local translation. We previously identified microtubule-dependent mobile vesicles rich in SMN and SNRPB, a member of the Sm family of small nuclear ribonucleoprotein (snRNP)-associated proteins, in neural cells. By comparing the interactomes of SNRPB and SNRPN, a neural-spe  ...[more]

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