Endosomal accumulation of APP in wobbler motor neurons reflects impaired vesicle trafficking: Implications for human motor neuron disease

Table 1 APP⁺ vesicle formation in MND autopsies

CASE #	AGE	GENDER	PM [h]	PATHOLOGICAL DIAGNOSIS	DEG.	APP+ VESICLES	VPS54 SEQ. ALTERATION
1	74	M	83	MND	-3	+	n.d.
2	49	M	32	MND	-3	-	None
3	58	M	37	MND	-2	-	n.d.
4	69	F	17	MND	-3	+	n.d.
5	48	M	25	MND	-2	+	None
6	81	F	30	MND	-2	-	None
7	55	M	39	MND	-2	-	None
8	72	F	6	MND	-1	-	n.d.
9	68	F	70	MND	-3	+	None
10	54	M	30	MND	-1	-	n.d.
11	51	F	24	MND	-3	+	None
12	54	M	95	MND	-2	+	n.d.
13	54	F	?	MND Familial	-3	-	n.d.
14	56	M	79	MND Familial	-2	-	n.d.
15	89	F	54	PD	-1	-	None
16	85	F	9	PD	-2	-	None
17	61	M	65	Amyloid plaques	0	-	n.d.
18	79	M	24	Mild ageing changes	0	-	n.d.
19	80	M	48	changes consistent with ageing	0	-	n.d.
20	40	M	40	Normal adult brain	0	-	n.d.
21	57	M	26	Normal adult brain	0	-	n.d.
22	87	F	21	Normal adult brain	0	-	None
23	68	M	53	Normal adult brain	0	-	n.d.
24	64	F	60	Normal adult brain	0	-	n.d.
25	61	M	35	Normal adult brain	0	-	n.d.
26	58	F	21	Mild ischemia	0	-	None
27	68	M	40	Diffuse hypoxic damage	0	-	n.d.
28	61	M	65	None given	0	-	n.d.

Legend: Histological assessment of human Motor neuron disease (MND) autopsies from spinal cord. Spinal cord sections were fixed in formalin after the post-mortem time indicated ("PM", in hours [h]) and later embedded in paraffin. The grades of Motor neuron degeneration were judged by Nissl staining and designated as 0 (no degeneration), -1 (single degenerating neurons), -2 (more than one degenerating motor neuron per ventral horn), -3 (groups of degenerating motor neurons). Note that in non-ALS brains including normal adult brain or other origins of neurodegeneration, APP⁺ vesicles have not been detected.

ISSN: 1471-2202