mmm appear to contain cloning artifacts. 13. The 5' end of the human cDNA was obtained by two rounds of PCR amplification of the CLN2 candidate from a human cortex cDNA library (Stratagene) by means of two different gene-specific primers and a single vector-specific primer. In the first round of PCR the T3 promoter primer was used with either gene-specific primer NR1 (5'-GT-GATCACAGAATGGCACTT) or NR2 (5'-AACAT-GGGTTTCCGTAGGTC). In the second round of PCR, with the products from the first amplification, the T3 promoter primer and NR4 (5'-CTTCCT-CAGGGTCCGCACGG) were used. 14. GenBank accession number AF017456. 15. Three lines of evidence give corroborative results for an unequivocal localization, (i) There is a nearly perfect match between nt 34 to 104 of the CLN2 cDNA candidate and GenBank accession number B04497, which represents a PCR-amplified fragment of a-flow-sorted chromosome 11-specific cosmid clone. (The 317-nt B04497 also contains sequence of flanking introns.) (ii) There is a perfect 505-nt match between the 3' end of the CLN2 cDNA (nt 2979 to 3483) and the 5' end (nt 1 to 505) of GenBank accession number U25816. U25816 consists of 2605 nt that encompass the human TATAbinding protein associated factor II 30 (TAFn30) gene. The TAFM30 transcription start site is at U25816 nt 1060, and most of the promoter elements are downstream of U25816 nt 860 and thus do not overlap with the 3' end of the large CLN2 candidate transcript. Thus, the CLN2 candidate gene and the TAFn30 gene are physically adjacent. The TAF,|30 gene was mapped to chromosome 11 p15.2-p15.5 by means of in situ hybridization [E.

Purpose To investigate the progression characteristics of ellipsoid zone (EZ) loss in eyes with macular telangiectasia type 2 (MacTel) as reflected by area and linear measurements, and their relevance for visual acuity. Methods Participants were selected from the MacTel Study cohort. Linear and area measurements of EZ loss were performed in Spectral‐Domain Optical Coherence Tomograph (SD‐OCT) volume scans. Progression characteristics and correlations between linear and area measurements were analysed using linear mixed effects models. Results A total of 134 eyes of 70 patients were included (85 eyes with follow‐up, mean 4.7 years, range: 1.4–8 years). Ellipsoid zone (EZ) loss significantly progressed at a mean annual increment of 0.057 mm2 (p = 0.005). The progression rate was non‐linear and interacted significantly with initial EZ lesion size indicating an exponential growth before reaching a plateau. There was a strong heterogeneity in area sizes between fellow eyes. EZ break length had a significant linear effect on EZ break area (b = 1.06, p < 0.001) and could predict it. The location of the EZ break had a significant impact on visual acuity. Conclusion Ellipsoid zone (EZ) loss in MacTel has a non‐linear progression characteristic, and its rate depends on area size at baseline, which must be taken into account at sample selection in clinical trials. Our results show a good correlation of linear and area measures of EZ loss and a segregation of best‐corrected visual acuity by EZ location, which may help routine clinical practice.

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