Table 1 Phenotypic comparison of the ΔLYST allele on the C57BL/6J and DBA/2J backgrounds, beige allele on the C57BL/6J and DBA/2J backgrounds, and Lyst^Ing3618 allele on the C57BL/6J background

Phenotype	Lyst^bg-J C57BL/6 J	Lyst^bg-J DBA/2 J	Lyst^Ing3618 C57BL/6 J	ΔLYST-B6 C57BL/6 J	ΔLYST-DBA DBA/2 J
Partial albinism	✓	✓	✓	✓	✓
Ophthalmic phenotype (retina or iris)^a	✓	✓	×	✓	Not tested
Hematological phenotype	✓	Not tested	✓	✓	Not tested
Immunological phenotype	✓	Not tested	×	Not tested	Not tested
Onset of neurological phenotype	>12 months (by CPSS)	>12 months (by CPSS)	12 months (by coat hanger and stationary beam test)	6 months (by composite ataxia score)	3 months (by composite ataxia score)
Onset of significant Purkinje cell loss	17-20 months	17–20 months	12 months¹	18 months	Not tested
Onset of significant peripheral neuropathy	Not tested	Not tested	12 months	3 months	Not tested
Reference	Hedberg-Buenz et al.¹⁰	Hedberg-Buenz et al.¹⁰, Tantrow et al. 2010	Rudelius et al.¹²	This study	This study

✓ present, × absent, CPSS composite phenotypic scoring system for mouse models of cerebellar ataxia (Guyenet et al. 2010); ¹Intracytoplasmic inclusions were observed by histological analysis starting at 5 months in the homozygous mutant mice. ^aNote, most studies focused on characterizing the retina, except in Tantrow et al, where the study focused on the iris.

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