Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.1.6.1 (sulfatase)
 3,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sulfatase modifying factor 1 (SUMF1) encodes for the formylglicine generating enzyme, which activates sulfatases by modifying a key cysteine residue within their catalytic domains. SUMF1 is mutated in patients affected by multiple sulfatase deficiency, a rare recessive disorder in which all sulfatase activities are impaired. Despite the absence of canonical retention/retrieval signals, SUMF1 is largely retained in the endoplasmic reticulum (ER), where it exerts its enzymatic activity on nascent sulfatases. Part of SUMF1 is secreted and paracrinally taken up by distant cells. Here we show that SUMF1 interacts with protein disulfide isomerase (PDI) and ERp44, two thioredoxin family members residing in the early secretory pathway, and with ERGIC-53, a lectin that shuttles between the ER and the Golgi. Functional assays reveal that these interactions are crucial for controlling SUMF1 traffic and function. PDI couples SUMF1 retention and activation in the ER. ERGIC-53 and ERp44 act downstream, favoring SUMF1 export from and retrieval to the ER, respectively. Silencing ERGIC-53 causes proteasomal degradation of SUMF1, while down-regulating ERp44 promotes its secretion. When over-expressed, each of three interactors favors intracellular accumulation. Our results reveal a multistep control of SUMF1 trafficking, with sequential interactions dynamically determining ER localization, activity and secretion.
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PMID:Multistep, sequential control of the trafficking and function of the multiple sulfatase deficiency gene product, SUMF1 by PDI, ERGIC-53 and ERp44. 1850 57

Powdery mildew, caused by Blumeria graminis f. sp tritici (Bgt) is one of the devastating diseases of wheat and causes yield losses in temperate wheat growing regions. A wheat line, N0308 with resistance to powdery mildew was used in this study. A suppression subtractive hybridization cDNA library was constructed from the wheat leaves inoculated by Bgt at the two-leaf stage. The differentially expressed genes in response to Bgt infection in wheat were identified, and a total of 175 positive clones from the library were sequenced, and 90 expressed sequence tags (ESTs) were subjected to clustering, BLAST alignment, functional annotation, and classification into different categories. By comparing the EST sequences among the SSH-cDNA libraries, we analyzed gene expression patterns of 7 ESTs associated with the resistance reaction of powdery mildew by using semi-quantitative reverse transcription-polymerase chain reaction. The expression of 5 genes (sulfatase, pathogenesis-related protein 17, betacarbonic anhydrase 2, thioredoxin h-like protein, and coronatine-insensitive) transcripts was induced, and the transcript levels of these genes were the highest at 72 h after Bgt infection, while those of 2 genes (violaxanthin de-epoxidase and gag-pol-polyprotein) were the highest level at 12 and 18 h post-infection, respectively. These findings suggest that these genes are induced at an early stage of infection and are transcriptionally activated for the host defense response.
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PMID:Differential expression of resistance to powdery mildew at the early stage of development in wheat line N0308. 2503 73