Table 1 Clinical Data Regarding 3 Subjects with Wolfram Syndrome with the Highest Levels of PSP/reg.

From: Pancreatic stone protein/regenerating protein is a potential biomarker for endoplasmic reticulum stress in beta cells

Subject

Age

Gender

PSP/reg (ng/mL)

Fasting

WFS1

Age of disease onset

Glucose (mg/dL)

C-peptide (ng/mL)

Allele 1

Allele 2

DM

OA

Hearing loss

DI

WU.WOLF-03

22

M

94.7

279

0.65

c.1230_1233delCTCT; p.V412fs*440

c.1243_1245delGTC; pV415del

4

6

6

6

WU.WOLF-11

12

M

126.8

278

0.4

c.376G>A; p.A126T

c.1838G>A; p.W613*

7

7

8

8

WU.WOLF-12

26

M

67.3

195

0.14

c.320G>A; p.G107E

c.1882C>T; p.R629W

6

7

7

15

  1. Subject number, age, gender, and genotype of these subjects. Age (years) of clinical onset of DM (diabetes mellitus), OA (optic atrophy), hearing loss, DI (diabetes insipidus). Also listed is the subjects fasting PSP/reg, glucose, and C-peptide levels.
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