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Two novel variants in the PSAP gene were identified in a patient with metachromatic leukodystrophy.
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Saposin C or its precursor (PSAP) function as an androgen-agonist and upregulate androgen receptor and prostate-specific antigen expression/activity in androgen-responsive prostate cancer cells LNCaP, TRAMP-C1 and -C2, and CWR 22RV1.
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Prosaposin is an androgen-target gene and its expression in androgen-responsive prostate cancer cells can be upregulated by male hormones.
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The prosaposin gene is amplified in human metastatic AI PCa cell lines and punch biopsy samples of prostate cancer xenografts and lymph node metastases.
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The findings of this study suggest that PSAP can promote glioma cell proliferation via the TLR4/NF-kappaB signaling pathway and may be an important target for glioma treatment.
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Accumulating evidence suggests that PGRN is essential for proper lysosomal function, but the precise mechanisms involved are not known. Here, we show that PGRN facilitates neuronal uptake and lysosomal delivery of prosaposin (PSAP), the precursor of saposin peptides that are essential for lysosomal glycosphingolipid degradation.
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Both PSAP reduction and overexpression lead to significantly elevated extracellular progranulin (PGRN) levels. Intriguingly, PSAP knockdown increases PGRN monomers, whereas PSAP overexpression increases PGRN oligomers, partly through a protein-protein interaction.
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Study demonstrated that the binding of CST3 and PSAP decreased the inhibitory effects of CST3 on proteinase in vitro. The co-localization of both proteins was detected in cultured cells and in Bunina body-containing motor neurons from patients with amyotrophic lateral sclerosis, suggesting that they might be involved in the process of Bunina body formation.
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This report documents the successful use of plasma lysoSLs profiling in the PSAP deficiency diagnosis, as a reliable and informative tool to obtain a preliminary information in infantile cases with complex traits displaying severe neurological signs and visceral involvement.
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an extensive review of all the PSAP-causative variants published in the literature to date, accounting for a total of 10 PSAP allele types (review)
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Our findings suggest a novel pharmacological approach to Sap C deficiency directed to treat major secondary pathological aspects in this disorder.
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PSAP is a secreted biomarker.
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Data suggested that the abundance of Psap in sperm sample may be a sensitive endpoint to predict PCB exposure.
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Prosaposin facilitates sortilin-independent lysosomal trafficking of progranulin.
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findings support a lung metastasis-promoting function of the miR-23b/27b/24 cluster of miRNAs, which functions in part through the direct inhibition of PSAP in breast cancer
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Mesotrypsin generated saposins A-D from prosaposin, and mature caspase-14 contributed to this process by activating mesotrypsinogen to mesotrypsin. Knockdown of these proteases markedly down-regulated saposin A synthesis in skin equivalent models.
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Of the 2575 proteins identified, proteins upregulated in gallbladder cancer included several lysosomal proteins such as prosaposin, cathepsin Z and cathepsin H.
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Saposin C protects glucocerebrosidase against alpha-synuclein inhibition.
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Urine of patients with early prostate cancer contains lower levels of light chain fragments of inter-alpha-trypsin inhibitor and prosaposin fragment or saposin B.
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These findings suggested that prosaposin might enhance estrogen receptor alpha-mediated signaling axis and play a role in breast cancer development and progression.
