. Author manuscript; available in PMC: 2018 Jan 1.

Published in final edited form as: Mol Genet Metab. 2016 Oct 11;120(1-2):101–110. doi: 10.1016/j.ymgme.2016.10.001

Table 1. Summary of proteins identified by LC-MS/MS from the picked 2D gel spots which are differentially expressed between MPS I and wildtype mice.

Positive fold changes represent an up-regulation while negative values signify a down-regulation of protein expression in MPS I versus wildtype.

Spot#	Protein name	Mascot score	# of peptides	NCBI accession	Protein MW (kD)	Fold change (MPS vs WT)	T-test (p)
81	murinoglobulin-1 precursor	29	1	gi\|31982171	166968	−5.1	0.019
	oxidation resistance protein 1 isoform E	28	10	gi\|194328708	96421
	ryanodine receptor 3	27	5	gi\|125628627	554439
149	drebrin A	37	8	gi\|6694227	78136	4.1	0.009
254	alpha-actinin-4 isoform X2	76	4	gi\|568946338	104880	−3.7	0.013
254	dynamin/mKIAA4093 protein	68	9	gi\|60360130	98009
285	glucosidase 2 subunit beta isoform 2 precursor	39	2	gi\|6679465	59963	3.8	0.023
	unnamed protein product	27	1	gi\|74193788	27642
	phosphatidylcholine-specific phospholipase D1b	27	2	gi\|2541940	120066
358	dual specificity phosphatase-like 15, isoform CRA_b, partial	29	2	gi\|148674056	10754	3.8	0.022
	Col4a6 protein	20	2	gi\|34785873	107153
	epsin-1	29	1	gi\|46195711	60317
583	unc-18 homologue/Munc18-1	83	3	gi\|1944322	68156	−6.6	0.043
583	dihydropyrimidinase-related protein 2	65	7	gi\|40254595	62736
591	dihydropyrimidinase-related protein 2/Ulip2 protein	276	24	gi\|40254595	62736	−5.0	0.013
	unc-18 homologue/Munc18-1	95	10	gi\|3810884	68004
	FERM domain containing 3, isoform CRA_a, partial	30	1	gi\|148699106	67284
609	dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial	70	9	gi\|257796245	68609	−5.5	0.014
	heat shock-related protein	46	2	gi\|957195	71148
	dihydropyrimidinase-related protein 2/Ulip2 protein	33	4	gi\|40254595	62736
613	dihydropyrimidinase-related protein 2/Ulip2 protein	106	23	gi\|40254595	62736	−4.6	0.011
613	unc-18 homologue/Munc18-1	58	3	gi\|1944322	68156
615	dihydropyrimidinase-related protein 2/Ulip2 protein	78	22	gi\|40254595	62736	−4.2	0.001
	dihydropyrimidinase-related protein 4 isoform X2	48	4	gi\|568952370	34187
	glutaminase kidney isoform, mitochondrial isoform X2	36	2	gi\|568905794	50243
644	dihydropyrimidinase-related protein 2/Ulip2 protein	44	11	gi\|40254595	62736	−5.0	0.017
644	chaperonin containing TCP-1 theta subunit	34	2	gi\|5295992	60170
720	dihydropyrimidinase-related protein 2/Ulip2 protein	51	10	gi\|40254595	62736	−3.8	0.026
720	mKIAA3028 protein	23	4	gi\|50511271	189949
906	actin, alpha skeletal muscle	43	4	gi\|4501881	42450	−3.8	0.018
	perinuclear binding protein	41	1	gi\|577078	47127
	tubulin beta-4B chain	35	4	gi\|5174735	50367
919	V-type proton ATPase subunit B, brain isoform	132	12	gi\|17105370	56941	−4.2	0.007
	MNCb-1930 protein/cytosolic non-specific dipeptidase	53	7	gi\|12697592	53274
	PHD finger protein 24	48	2	gi\|27369996	42227
	spliceosome RNA helicase Ddx39b	41	2	gi\|9790069	49572
	solute carrier family 9 (sodium/hydrogen exchanger), isoform 3 regulator 1, isoform CRA_b, partial	36	10	gi\|148702516	32049
947	PHD finger protein 24 isoform X2	40	4	gi\|1039767264	47318	−8.7	0.012
947	Na(+)/H(+) exchange regulatory cofactor NHE-RF1	30	3	gi\|6755566	38932
1088	NADP-dependent isocitrate dehydrogenase	37	1	gi\|3641400	47128	3.6	0.004
	erythrocyte protein band 4.9, isoform CRA_a	30	1	gi\|148703936	36082
	SEC14-like protein 2	31	3	gi\|21362309	46839
1162	Melanocyte proliferating gene 1	36	3	gi\|20306404	43166	−4.0	0.027
	UPF0160 protein MYG1, mitochondrial precursor	35	5	gi\|11096332	43193
	heterogeneous nuclear ribonucleoprotein A/B isoform 2	32	4	gi\|6754222	30954
1206	leukocyte elastase inhibitor A	131	6	gi\|114158675	42761	−4.2	0.042
1226	fructose-bisphosphate aldolase A isoform 2	40	5	gi\|6671539	39900	−14.1	0.021
1226	guanine nucleotide-binding protein G(o) subunit alpha isoform A	24	6	gi\|6754012	40769
1351	SIR2L2	119	8	gi\|11141704	44026	−5.7	0.013
	poly(rC)-binding protein 1	3	32	gi\|6754994	38113
	acyl-CoA hydrolase	30	1	gi\|14587839	38028
	fructose-bisphosphate aldolase A isoform 2	29	2	gi\|6671539	39900
1575	pyridoxal phosphate phosphatase	344	19	gi\|47059486	31989	−3.6	0.042
1575	immunoglobulin variable region	27	2	gi\|288821	13708
1613	14-3-3 protein theta	80	2	gi\|6756039	28116	4.7	0.033
	14-3-3 zeta	79	3	gi\|1526539	27950
	isoamyl acetate-hydrolyzing esterase 1 homolog ]	38	1	gi\|27754071	28524
1636	mCG145112, partial	28	1	gi\|148676777	12544	4.9	0.019
1636	glutamate receptor channel subunit epsilon 4	28	1	gi\|2160438	144523
1669	MRJ	68	2	gi\|3142372	26995	−4.1	0.017
	glutathione S-transferase Mu 2	35	1	gi\|6680121	25913
	triosephosphate isomerase	29	3	gi\|54855	27105
	plexin 2	34	2	gi\|1655432	214098
	triosephosphate isomerase	30	5	gi\|54855	27105
1889	alpha-crystallin B chain	36	2	gi\|6753530	20056	−6.8	0.007