Disease relevance of PECI

• METHODS: One-hundred forty-nine parents of patients (age <1-17 years) diagnosed with a brain tumor were assessed using the 25-item self-report Parent Experience of Child Illness (PECI) [1].
• CONCLUSION: The PECI augments the current literature by providing a brief measure of parents' subjective distress and perceived Emotional Resources, domains that are critical but understudied in children with chronic illness and their caregivers [1].
• DRS-1 gene was abundantly expressed in myeloid leukemia cell lines and in CD34(+) cells derived from healthy individuals [2].
• Enzyme-linked immunosorbent assay (ELISA) revealed high titers of anti-DRS-1 antibodies (DRS-1 Abs) in 27 (38.0%) of 71 AA patients displaying increased paroxysmal nocturnal hemoglobinuria (PNH)-type cells (PNH(+)), 2 (6.3%) of 32 PNH(-) AA patients, 5 (38.5%) of 13 PNH(+) myelodysplastic syndrome (MDS) patients, and none of 42 PNH(-) MDS patients [2].
• To identify candidate antigens in aplastic anemia (AA), we screened proteins derived from a leukemia cell line with serum of an AA patient and identified diazepam-binding inhibitor-related protein 1 (DRS-1) [2].

High impact information on PECI

• These findings indicate that DRS-1 may serve as an autoantigen eliciting immune attack against hematopoietic stem cells in a subset of AA patients characterized by increased PNH-type cells [2].
• Enzyme-linked immunospot assay demonstrated increased frequency of T-cell precursors specific to the DRS-1 peptide in other HLA-DR15(+) AA patients displaying high DRS-1 Ab titers [2].
• Stimulation of T cells from an AA patient displaying high DRS-1 Abs with a putative CD4(+) T-cell epitope (amino acid residues [aa's] 191-204) presented by HLA-DR15, which overlapped with a hot spot (aa's 173-198) of DRS-1 Ab epitopes, gave rise to T cells cytotoxic for L cells (murine fibroblasts) that were transfected with DRB1*1501 and DRS-1 [2].
• The human and mouse PECI proteins contain type-1 peroxisomal targeting signals, and human PECI was localized to peroxisomes by both subcellular fractionation and immunofluorescence microscopy techniques [3].
• Cloning and sequencing of the human PECI cDNA revealed the presence of a 1077-base pair open reading frame predicted to encode a 359-amino acid protein with a mass of 39.6 kDa [3].

Biological context of PECI

• However, Southern blot analyses, in situ hybridizations, and somatic cell hybrid chromosomal mapping all suggest that there are multiple ACBP/DBI-related sequences in the genome [4].
• An anti-HA1 antibody could detect all four proteins in crude lysates and yielded the relative abundance of these four proteins, of which Drs1 is reproducibly less abundant than any of the others, which may have implications for the control of ribosome biogenesis [5].

Anatomical context of PECI

• Characterization of PECI, a novel monofunctional Delta(3), Delta(2)-enoyl-CoA isomerase of mammalian peroxisomes [3].
• DRS-1 was expressed in most tissues including liver, lung, tonsil, and thymus, in addition to pancreatic islets [6].
• These results indicate that, in the frog adrenal gland, DBI-related peptides and PBR are simultaneously expressed in chromaffin cells and Stilling s cells, suggesting that endogenous ligands for PBR may play a physiological role in the control of adrenal cell activity [7].

Analytical, diagnostic and therapeutic context of PECI

• Northern blot analysis demonstrated human PECI mRNA is expressed in all tissues [3].
• The disability rating scale at the time of discharge from the rehabilitation unit (DRS1) and at 1 year follow up (DRS2) were functional outcome measures [8].

References