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. Author manuscript; available in PMC: 2024 May 20.
Published in final edited form as: Rare. 2023 Dec 13;2:100017. doi: 10.1016/j.rare.2023.100017

Table 1.

Polyamine concentrations in fibroblast lysates were determined in duplicate following derivatization with dansyl chloride and HPLC analyses.

Sample ID Genotype PUT SPD SPM SPD/SPM
(nmol/mg Protein) ratio
Control 1 wt -- 4.18 5.85 0.715
-- 4.08 5.82 0.701
Control 2 wt -- 2.72 4.88 0.557
-- 2.36 4.84 0.488
Proband Sample 1 p.Y249C 0.71 10.42 3.04 3.428
0.64 10.38 3.05 3.403
Proband Sample 2 p.Y249C 1.51 11.29 3.81 2.963
1.44 11.44 3.87 2.956
Individual 1 with SRS p.M35R -- 8.02 1.85 4.335
-- 8.02 1.86 4.312
Individual 2 with SRS P.L277F -- 9.06 2.16 4.194
-- 9.11 2.13 4.277

The spermidine/spermine ratio was compared to two sex-matched wildtype (wt) cell lines and two previously identified SRS lines. Polyamine levels were normalized according to protein concentration, with 1,7-diaminoheptane as the internal standard. PUT = putrescine, SPD = spermidine, SPM = spermine.