Lysosomal Storage Disease malady

Disease Ontology: An inherited metabolic disorder that involve an abnormal accumulation of substances inside the lysosome resulting from defects in lysosomal function.⁶

MalaCards: Lysosomal Storage Disease, also known as lysosomal storage diseases, is related to mucopolysaccharidosis vii and beta-mannosidosis. An important gene associated with Lysosomal Storage Disease is AGA (aspartylglucosaminidase), and among its related pathways are Galactose metabolism and Glycosphingolipid biosynthesis - globo series. The compounds (z)-pugnac and n-acetylgalactosamine 6-sulfate have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, brain and t cells, and related mouse phenotypes are limbs/digits/tail and hematopoietic system.

Wikipedia: Lysosomal storage diseases (LSDs; pron.: /ˌlaɪsəˈsoʊməl/) are a group of approximately 50 rare...⁴⁴ more...

Aliases & Descriptions:

lysosomal storage disease 6 8 lysosomal storage diseases 17 32 43 disorder of lysosomal enzyme (disorder) 6

lysosomal storage metabolism disorder 6 inborn lysosomal enzyme disorder 6

Diseases related to lysosomal storage disease by text searches and GeneDecks gene sharing:

(show top 50)    (show all 111)

id	Related Disease	Score	Top Affiliating Genes
1	mucopolysaccharidosis vii	32.8	GUSB, IDUA
2	beta-mannosidosis	30.7	SGSH, NAGA, MANBA
3	mucopolysaccharidosis iii	30.6	SGSH, NAGLU, HGSNAT
4	schindler disease	30.5	NAGA, GLA
5	galactosialidosis	30.4	GLA, GLB1, CTSA, NEU1
6	fucosidosis	30.4	NAGA, FUCA1, GLA, HEXA, AGA
7	acid sphingomyelinase deficiency	30.4	M6PR, SMPD1, NPC1
8	lipid storage disease	30.3	LIPA, PSAP, GALC
9	mucopolysaccharidosis ii	29.9	IDS, HGSNAT
10	gaucher's disease	29.9	CHIT1, MRC1, GUSB, ARSH, ARSA, IDUA
11	sialidosis	29.9	NAGA, CHIT1, GLB1, CTSA, PSAP, SLC44A4
12	hydrops fetalis	29.9	GUSB, SLC17A5, NEU1, GBA
13	pick's disease	29.8	NAGA, CHIT1, M6PR, SMPD1, PSAP, NPC1
14	danon disease	29.6	LAMP2, GLA, GAA
15	metachromatic leukodystrophy	29.5	ARSH, ARSA, ARSB, GLA, SUMF1, HEXA
16	batten disease	29.4	CLN5, CLN6, CLN3, CLN8, TPP1, CTSD
17	fabry disease	29.3	NAGA, CHIT1, FUCA1, GUSB, ARSA, MANBA
18	neuronal ceroid-lipofuscinoses	29.2	CLN5, CLN6, CLN3, CLN8, TPP1, CTSD
19	mucopolysaccharidosis	28.7	SGSH, NAGLU, GUSB, ARSH, ARSA, ARSB
20	neurodegeneration	26.2	CLCN7, CLN3, CLN8, LAMP2, GUSB, ARSH
21	neuronitis	22.2	CLCN6, CLN5, CLN6, CLCN7, CLN3, CLN8
22	gaucher disease type 1	13.7	CHIT1, GBA
23	mucopolysaccharidosis i	13.7	ARSH, IDUA
24	mucolipidosis iv	13.6	MCOLN1, HEXA
25	gaucher disease type 2	13.6	PSAP, GBA
26	gaucher disease type 3	13.6	PSAP, GBA
27	craniofacial abnormalities	13.6	GUSB, ARSB, IDUA
28	mucopolysaccharidosis vi	13.5	ARSH, ARSB, GALNS
29	hurler syndrome	13.5	IDUA, GLB1
30	kanzaki disease	13.5	NAGA, GLA
31	mucosulfatidosis	13.5	ARSH, ARSA, ARSB, GALNS
32	progressive myoclonus epilepsy	13.5	CLN5, CLN3, TPP1, GBA
33	angiokeratoma	13.5	NAGA, FUCA1, MANBA, GLA, CTSA, AGA
34	niemann-pick disease type c1	13.4	SMPD1, PSAP, NPC1
35	mannosidosis	13.4	SGSH, NAGA, MAN2B1, MANBA
36	ceroid lipofuscinosis neuronal 8	13.4	CLN5, CLN3, CLN8
37	dysostosis	13.4	FUCA1, ARSA, MAN2B1, NEU1
38	corneal clouding	13.4	GUSB, ARSB, IDUA, GLB1, GALNS
39	mucopolysaccharidosis iv	13.4	GUSB, ARSH, GLB1, CTSA, NEU1, GALNS
40	mucolipidoses	13.4	GUSB, HYAL1
41	visual seizure	13.4	CLN6, CLN8, TPP1
42	chondrodysplasia punctata	13.4	ARSH, ARSA, ARSB, SUMF1
43	pelvic inflammatory disease	13.3	GUSB, CTSA, CTSB
44	niemann–pick disease	13.3	CHIT1, M6PR, SMPD1, PSAP, NPC1
45	tay-sachs disease	13.3	GM2A, HEXA, HEXB, PSAP, NEU1
46	iga glomerulonephritis	13.3	NAGA, NAGLU, GLA, NEU1
47	myoclonus epilepsy	13.3	CLN5, CLN3, TPP1, CTSB, GBA
48	fanconi syndrome	13.3	NAGLU, CTNS, CTSD
49	ichthyosis	13.3	ARSH, ARSA, ARSB, SUMF1, GBA
50	hydrocephalus	13.3	ARSH, ARSA, ARSB, SUMF1, GBA

Approved drugs:

Drug clinical trials:

Cell-based therapeutics:

MalaCards organs/tissues related to lysosomal storage disease:

²²

MGI Mouse Phenotypes related to lysosomal storage disease:

²⁵ (show all 20)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	limbs/digits/tail phenotype	MP:0005371	9.1	HEXB, HEXA, SUMF1, IDUA, IDS, ARSB
2	hematopoietic system phenotype	MP:0005397	9.0	HEXB, PPT1, CTSA, SUMF1, GLB1, MANBA
3	respiratory system phenotype	MP:0005388	8.5	GBA, NEU1, NPC1, PSAP, SMPD1, CTSA
4	hearing/vestibular/ear phenotype	MP:0005377	8.4	SLC17A5, PSAP, DNAJC5, HEXB, HEXA, IDUA
5	muscle phenotype	MP:0005369	8.0	GAA, GALC, NEU1, PSAP, DNAJC5, HEXB
6	cardiovascular system phenotype	MP:0005385	8.0	CTSA, PSAP, NEU1, GBA, GALC, GAA
7	liver/biliary system phenotype	MP:0005370	7.9	GALC, HYAL1, IDUA, MANBA, MAN2B1, CLN3
8	reproductive system phenotype	MP:0005389	7.8	GLB1, M6PR, SMPD1, DNAJC5, PSAP, NPC1
9	craniofacial phenotype	MP:0005382	7.7	SUMF1, CTSA, PPT1, HEXA, HEXB, OSTM1
10	renal/urinary system phenotype	MP:0005367	7.5	GALNS, GALC, NEU1, PSAP, AGA, HEXB
11	integument phenotype	MP:0010771	7.3	TPP1, CTSA, CTSB, AGA, OSTM1, NEU1
12	skeleton phenotype	MP:0005390	6.6	PSAP, GUSB, LAMP2, CLCN7, NAGLU, SGSH
13	vision/eye phenotype	MP:0005391	6.1	GALNS, GALC, NEU1, SLC17A5, NPC1, CLN3
14	homeostasis/metabolism phenotype	MP:0005376	5.1	DNAJC5, AGA, HEXB, HEXA, SMPD1, PPT1
15	immune system phenotype	MP:0005387	4.9	CTSD, CTSB, PPT1, SMPD1, SKAP2, HEXB
16	growth/size phenotype	MP:0005378	4.8	DNAJC5, AGA, HEXB, HEXA, SMPD1, CTSB
17	mortality/aging	MP:0010768	4.3	HEXB, HEXA, SMPD1, PPT1, CTSB, CTSD
18	cellular phenotype	MP:0005384	4.2	GAA, GALNS, GALC, GBA, NEU1, SLC17A5
19	nervous system phenotype	MP:0003631	3.8	LAMP2, CLN8, CLN3, CLCN7, CLN6, CLN5
20	behavior/neurological phenotype	MP:0005386	3.5	SLC17A5, NPC1, PSAP, OSTM1, DNAJC5, AGA

Articles related to lysosomal storage disease:

(show all 32)

id	Title	Authors	Year	Affiliating Genes
1	Biotherapeutic target or sink: analysis of the macrop hage mannose receptor tissue distribution in murine models of lysosomal storage diseases. (21416197)	Zhang X.S.... Piepenhagen P.A.	2011	MRC1
2	Mass spectrometry-based protein profiling to determin e the cause of lysosomal storage diseases of unknown etiology. (19383612)	Sleat D.E.... Lobel P.	2009	CLN5
3	Immune response hinders therapy for lysosomal storage diseases. (18654672)	Ponder K.P.	2008	IDUA
4	Lysosomal storage diseases and the blood-brain barrier. (18673198)	Begley D.J.... Scarpa M.	2008	M6PR
5	Non-inhibitory antibodies impede lysosomal storage re duction during enzyme replacement therapy of a lysosomal storage disease. (18360747)	Matzner U.... Gieselmann V.	2008	ARSA
6	Topology and endoplasmic reticulum retention signals of the lysosomal storage disease-related membrane protein CLN6. (17453415)	Heine C.... Braulke T.	2007	CLN6
7	Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6. (16950870)	Poet M.... Jentsch T.J.	2006	CLCN6
8	Functional correction of CNS phenotypes in a lysosomal storage disease model using adeno-associated virus type 4 vectors. (16221840)	Liu G.... Davidson B.L.	2005	GUSB
9	Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration. (15706348)	Kasper D.... Jentsch T.J.	2005	CLCN7
10	Widespread gene transduction to the central nervous system by adenovirus in utero: implication for prenatal gene therapy to brain involvement of lysosomal storage disease. (15459963)	Shen J.S.... Eto Y.	2004	GUSB
11	Human CD34+ hematopoietic progenitor cell-directed lentiviral-mediated gene therapy in a xenotransplantation model of lysosomal storage disease. (15194052)	Hofling A.A.... Sands M.S.	2004	GUSB
12	Determination of oligosaccharides and glycolipids in amniotic fluid by electrospray ionisation tandem mass spectrometry: in utero indicators of lysosomal storage diseases. (15542394)	Ramsay S.L.... Meikle P.J.	2004	PSAP, ARSH
13	CLN6, which is associated with a lysosomal storage disease, is an endoplasmic reticulum protein. (15265688)	Mole S.E.... Cutler D.F.	2004	CLN6
14	Engraftment of human CD34+ cells leads to widespread distribution of donor-derived cells and correction of tissue pathology in a novel murine xenotransplantation model of lysosomal storage disease. (12406886)	Hofling A.A.... Sands M.S.	2003	GUSB
15	Biodistribution and efficacy of donor T lymphocytes in a murine model of lysosomal storage disease. (12573618)	Young P.P.... Sands M.S.	2003	GUSB
16	Abnormal osteoclast morphology and bone remodeling in a murine model of a lysosomal storage disease. (11856642)	Monroy M.A.... Sands M.S.	2002	GUSB
17	Efficacy of gene therapy for a prototypical lysosomal storage disease (GSD-II) is critically dependent on vector dose, transgene promoter, and the tissues targeted for vector transduction. (11945071)	Ding E.... Amalfitano A.	2002	GAA
18	Non-viral, integrin-mediated gene transfer into fibroblasts from patients with lysosomal storage diseases. (11601762)	Estruch E.J.... Winchester B.G.	2001	FUCA1
19	Treatment of a lysosomal storage disease, mucopolysaccharidosis VII, with microencapsulated recombinant cells. (11044913)	Ross C.J.... Chang P.L.	2000	GUSB
20	Gene therapy/cell therapy for lysosomal storage disease. (10863945)	Eto Y.... Ohashi T.	2000	GUSB
21	The neuronal ceroid-lipofuscinoses (Batten disease): a new class of lysosomal storage diseases. (10407785)	Bennett M.J.... Hofmann S.L.	1999	PPT1, CLN3, CLN5
22	A model of mRNA splicing in adult lysosomal storage disease (glycogenosis type II). (8817337)	Raben N.... Plotz P.H.	1996	SV2A
23	Acid sphingomyelinase-deficient mice mimic the neurovisceral form of human lysosomal storage disease (Niemann-Pick disease). (7600574)	Otterbach B.... Stoffel W.	1995	SMPD1
24	Correction of deficient enzyme activity in a lysosomal storage disease, aspartylglucosaminuria, by enzyme replacement and retroviral gene transfer. (7548272)	Enomaa N.... Jalanko A.	1995	AGA
25	Medicinal therapy for lysosomal storage diseases (8577061)	Sakuragawa N.	1995	SMPD1
26	Fibroblast expression of collagens and proteoglycans is altered in aspartylglucosaminuria, a lysosomal storage disease. (8312372)	Maatta A.... Penttinen R.P.	1994	AGA
27	Behavioural abnormalities in a murine model of a human lysosomal storage disease. (8513128)	Chang P.L.... Pisa M.A.	1993	GUSB
28	Quantitative correlation between the residual activity of beta-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease. (1348043)	Leinekugel P.... Sandhoff K.	1992	ARSA
29	Lysosomal storage diseases presenting as transient or persistent hydrops fetalis. (1799421)	Bonduelle M.... Liebaers I.	1991	GUSB
30	Lysosomal storage diseases. (1883197)	Neufeld E.F.	1991	IDUA
31	Diagnosis of lysosomal storage diseases. Pathomorphologic and biochemical possibilities (2122616)	August C.H.... Zschiesche M.	1990	HEXA
32	A method for the rapid detection of urinary glycopept ides in alpha-N-acetylgalactosaminidase deficiency and other lysosomal storage diseases. (2208741)	Schindler D.... Desnick R.J.	1990	NAGA

Genes related to lysosomal storage disease according to GeneCards:

(show top 50)    (show all 55)

id	Symbol	Description	Score	GeneCards Section Context
1	AGA	aspartylglucosaminidase	11.1	Publications, Summaries
2	SGSH	N-sulfoglucosamine sulfohydrolase	11.1	Summaries
3	FUCA1	fucosidase, alpha-L- 1, tissue	11.1	Publications, Summaries
4	GBA	glucosidase, beta, acid	11.1	Summaries
5	NEU1	sialidase 1 (lysosomal sialidase)	11.1	Summaries
6	HGSNAT	heparan-alpha-glucosaminide N-acetyltransferase	11.1	Summaries
7	MANBA	mannosidase, beta A, lysosomal	11.1	Summaries
8	CTNS	cystinosin, lysosomal cystine transporter	11.0
9	CLN3	ceroid-lipofuscinosis, neuronal 3	11.0	Orthologs, Publications
10	M6PR	mannose-6-phosphate receptor (cation dependent)	11.0	Publications
11	IDUA	iduronidase, alpha-L-	11.0	Publications
12	NAGA	N-acetylgalactosaminidase, alpha-	11.0	Publications
13	MCOLN1	mucolipin 1	11.0
14	CTSA	cathepsin A	11.0
15	TPP1	tripeptidyl peptidase I	11.0
16	HEXA	hexosaminidase A (alpha polypeptide)	11.0	Publications
17	IDS	iduronate 2-sulfatase	11.0
18	GUSB	glucuronidase, beta	11.0	Publications
19	ARSA	arylsulfatase A	11.0	Publications
20	HEXB	hexosaminidase B (beta polypeptide)	11.0
21	PPT1	palmitoyl-protein thioesterase 1	11.0	Publications
22	ARSH	arylsulfatase family, member H	11.0	Publications
23	SMPD1	sphingomyelin phosphodiesterase 1, acid lysosomal	11.0	Publications
24	PSAP	prosaposin	11.0	Publications
25	GAA	glucosidase, alpha; acid	11.0	Publications
26	GALNS	galactosamine (N-acetyl)-6-sulfate sulfatase	11.0
27	GLB1	galactosidase, beta 1	11.0
28	CHIT1	chitinase 1 (chitotriosidase)	11.0
29	ARSB	arylsulfatase B	11.0
30	ADPRH	ADP-ribosylarginine hydrolase	11.0
31	NAGLU	N-acetylglucosaminidase, alpha	11.0
32	SUMF1	sulfatase modifying factor 1	11.0
33	CTSD	cathepsin D	11.0
34	NPC1	Niemann-Pick disease, type C1	11.0
35	LIPA	lipase A, lysosomal acid, cholesterol esterase	11.0
36	GALC	galactosylceramidase	11.0
37	GLA	galactosidase, alpha	11.0
38	CTSB	cathepsin B	11.0
39	SLC44A4	solute carrier family 44, member 4	11.0	Summaries
40	CLN5	ceroid-lipofuscinosis, neuronal 5	11.0	Publications
41	CLN6	ceroid-lipofuscinosis, neuronal 6, late infantile, variant	11.0	Publications
42	CLN8	ceroid-lipofuscinosis, neuronal 8 (epilepsy, progressive with mental retardation)	11.0
43	HYAL1	hyaluronoglucosaminidase 1	11.0
44	GNS	glucosamine (N-acetyl)-6-sulfatase	11.0
45	GM2A	GM2 ganglioside activator	11.0
46	SV2A	synaptic vesicle glycoprotein 2A	11.0	Publications
47	MRC1	mannose receptor, C type 1	11.0	Publications
48	MAN2B1	mannosidase, alpha, class 2B, member 1	11.0
49	CLCN7	chloride channel, voltage-sensitive 7	11.0	Publications
50	DNAJC5	DnaJ (Hsp40) homolog, subfamily C, member 5	11.0

Pathways related to lysosomal storage disease according to GeneDecks:

(show all 11)

id	Pathway	Score	Top Affiliating Genes
1	Galactose metabolism20	10.0	GLA, GLB1, GAA
2	Glycosphingolipid biosynthesis - globo series20	9.9	HEXB, HEXA, GLA, NAGA
3	CS/DS degradation38	9.9	HEXB, HEXA, HYAL1, IDUA, IDS, ARSB
4	Glycosphingolipid biosynthesis - ganglio series20	9.8	GLB1, HEXA, HEXB
5	Sphingolipid metabolism20	9.7	GALC, GBA, NEU1, SMPD1, GLB1, GLA
6	Other glycan degradation20	9.7	GBA, NEU1, AGA, HEXB, HEXA, GLB1
7	MPS IIIC - Sanfilippo syndrome C38	9.6	GALNS, HGSNAT, GLB1, GNS, GUSB, NAGLU
8	Glycosaminoglycan degradation20	9.0	IDS, ARSB, GUSB, NAGLU, SGSH, IDUA
9	Metabolism of lipids and lipoproteins38	8.9	GALC, GBA, NEU1, SLC44A4, PSAP, SMPD1
10	Metabolic pathways20	8.2	SMPD1, HEXA, HEXB, HGSNAT, GBA, GALC
11	Lysosome20	4.2	GAA, GALNS, GALC, GBA, HGSNAT, NEU1

Compounds related to lysosomal storage disease according to GeneDecks:

(show top 50)    (show all 74)

id	Compound	Score	Top Affiliating Genes
1	(z)-pugnac42	10.6	HEXB, NAGLU
2	n-acetylgalactosamine 6-sulfate32	10.5	GALNS, ARSA, ARSH
3	4-methylumbelliferyl-beta-d-glucoside32	10.5	GALC, GBA, PSAP
4	miglustat32 9 9	12.4	CHIT1, GBA, GAA
5	dermatan32 18	11.4	IDUA, IDS, ARSB, GUSB
6	1-deoxynojirimycin32 42 9 9	13.4	GAA, GBA, NEU1, GLA, FUCA1
7	formylglycine32	10.4	SUMF1, ARSB, ARSA, ARSH
8	mucopolysaccharide32	10.3	IDUA, IDS, ARSB, ARSA, ARSH
9	N-Acetyl-D-glucosamine9 18 9	12.3	HEXB, HEXA, CHIT1, NAGLU
10	glucuronic acid32	10.3	GALNS, ADPRH, HEXA, ARSH, GUSB
11	dermatan sulfate32	10.2	GALNS, IDUA, IDS, ARSB, GUSB
12	cerebroside32	10.2	HEXA, GLA, ARSB, ARSA, ARSH
13	nh4cl32	10.2	NEU1, CTSB, CTSD, M6PR
14	glucosamine32 9 18 9	13.2	GALNS, ADPRH, NEU1, CTSD, ARSH
15	asparagine32	10.1	GBA, ADPRH, AGA, M6PR, ARSA
16	galactosylceramide32	10.1	GALC, PSAP, ARSA
17	sulfatide32	10.1	NEU1, PSAP, GLB1, GM2A, ARSA, ARSH
18	heparin32 9 18 9	13.0	GALNS, HEXA, CTSB, CTSD, IDUA, ARSH
19	chondroitin sulfate32 18	11.0	GALNS, NEU1, GLB1, HYAL1, ARSB, ARSH
20	chloroquine32 34 9 9	13.0	GAA, ADPRH, CTSB, CTSD, MCOLN1
21	ceramide32	10.0	GBA, PSAP, SMPD1, CTSD, GLA, ARSA
22	glycosaminoglycan32	9.9	GALNS, IDUA, IDS, ARSB, ARSA, ARSH
23	carbohydrates32	9.9	GAA, ADPRH, NEU1, ARSH, MRC1, NAGA
24	carbon32	9.8	ADPRH, PSAP, CTSB, GLA, FUCA1, NAGA
25	glycocholate32	9.8	ADPRH, SLC17A5, GUSB
26	sialic acid32	9.8	NEU1, SLC17A5, CTNS, ARSH, FUCA1, NAGLU
27	glucosylceramide32 18	10.7	GALC, GBA, PSAP, SMPD1, GLB1, GLA
28	heparan sulfate32 18	10.6	GALNS, HGSNAT, HEXA, M6PR, GNS, IDUA
29	sulfate32 18	10.6	IDUA, GNS, PSAP, GALNS, IDS, ARSB
30	glycolipid32	9.5	GALC, GM2A, GLA, HEXA, HEXB, PSAP
31	valine32	9.5	ADPRH, PSAP, CTSB, CTSD, GLA, ARSA
32	fluoride32	9.5	NAGLU, CTSD, SLC17A5, ADPRH
33	n-acetylglucosamine32	9.5	ARSB, GNS, CTSD, HEXA, HEXB, AGA
34	hyaluronic acid32 18	10.4	GALNS, NEU1, SLC17A5, CTSD, HYAL1, NAGLU
35	glycosphingolipid32	9.4	GBA, PSAP, SKAP2, CTSD, GLA, ARSA
36	ganglioside32	9.4	FUCA1, ARSA, IDUA, NEU1, GM2A, GLA
37	arginine32	9.4	GAA, GALNS, ADPRH, PSAP, SMPD1, CTSB
38	dmso32	9.2	CTSD, CTSB, SMPD1, SLC17A5, NEU1, GALNS
39	polysaccharide32	9.2	NEU1, ADPRH, GBA, GALNS, GAA, M6PR
40	methionine32	9.1	SLC17A5, SMPD1, CTSD, GLA, ARSA, FUCA1
41	lactate32	9.1	GAA, ADPRH, SLC17A5, CTSB, CTSD, GLA
42	hydrogen32 18	10.0	GBA, PSAP, ARSH, ARSA, GLA, SV2A
43	serine32	8.7	PPT1, SMPD1, HEXA, PSAP, NEU1, ADPRH
44	cholesterol32 9 18 9	11.3	SMPD1, PSAP, NPC1, SLC17A5, GBA, CTSB
45	mannose32	8.3	HEXB, NEU1, ADPRH, GBA, GAA, HEXA
46	cysteine32	8.1	SMPD1, AGA, DNAJC5, PSAP, NPC1, NEU1
47	aspartate32	8.1	GALNS, ADPRH, SLC17A5, AGA, SKAP2, SMPD1
48	calcium32 9 18 9	10.8	SUMF1, CTSD, CTSB, SMPD1, DNAJC5, PSAP
49	lipid32	7.4	M6PR, PPT1, SMPD1, HEXA, PSAP, NPC1
50	mannose 6-phosphate32 18	7.7	NAGLU, M6PR, TPP1, CTSA, CTSD, CTSB

Cellular components related to lysosomal storage disease according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	endoplasmic reticulum lumen	GO:005788	9.6	SUMF1, ARSB, ARSA, ARSH, CLN6
2	melanosome	GO:042470	9.6	TPP1, CTSD, CTSB, DNAJC5
3	endoplasmic reticulum	GO:005783	8.8	NPC1, AGA, CTSA, SV2A, CLN8, CLN3
4	lysosomal membrane	GO:005765	7.4	GAA, GBA, HGSNAT, NEU1, SLC17A5, NPC1
5	lysosomal lumen	GO:043202	7.4	GBA, NEU1, PSAP, HEXB, HEXA, SMPD1
6	lysosome	GO:005764	6.2	NAGA, GLA, GM2A, HYAL1, MANBA, MAN2B1

Biological processes related to lysosomal storage disease according to GeneDecks:

(show all 32)

id	Name	GO ID	Score	Top Affiliating Genes
1	lysosomal transport	GO:007041	10.4	ARSB, NPC1, HGSNAT
2	termination of signal transduction	GO:023021	10.3	GBA, SMPD1
3	ganglioside catabolic process	GO:006689	10.3	HEXB, HEXA, GM2A
4	sphingomyelin metabolic process	GO:006684	10.3	CLN3, SMPD1
5	ceramide biosynthetic process	GO:046513	10.3	GBA, SMPD1, CLN8
6	neuromuscular process controlling posture	GO:050884	10.3	GAA, HEXA, CLN8
7	glycolipid catabolic process	GO:019377	10.3	GM2A, NAGA
8	associative learning	GO:008306	10.2	PPT1, CLN8, CLN3
9	hyaluronan catabolic process	GO:030214	10.2	GUSB, HYAL1, HEXA, HEXB
10	hyaluronan metabolic process	GO:030212	10.2	GUSB, HYAL1, HEXA, HEXB
11	lipid storage	GO:019915	10.2	HEXB, HEXA, GM2A
12	protein deglycosylation	GO:006517	10.2	AGA, MAN2B1
13	lysosomal lumen acidification	GO:007042	10.2	CLN5, CLN6, CLN3, PPT1
14	sphingolipid catabolic process	GO:030149	10.1	PPT1, GM2A
15	receptor-mediated endocytosis	GO:006898	10.1	CLN3, MRC1, M6PR, PPT1
16	keratan sulfate catabolic process	GO:042340	10.1	GALNS, HEXB, HEXA, GLB1, GNS
17	keratan sulfate metabolic process	GO:042339	10.0	GALNS, HEXB, HEXA, GLB1, GNS
18	chondroitin sulfate catabolic process	GO:030207	10.0	HEXB, HEXA, HYAL1, IDUA, IDS, ARSB
19	chondroitin sulfate metabolic process	GO:030204	10.0	HEXB, HEXA, HYAL1, IDUA, IDS, ARSB
20	oligosaccharide catabolic process	GO:009313	10.0	HEXB, GM2A
21	lysosome organization	GO:007040	9.9	GAA, GBA, HEXB, HEXA, TPP1, ARSB
22	protein catabolic process	GO:030163	9.8	PPT1, TPP1, CLN8, CLN3, CLN6, CLN5
23	glycosaminoglycan catabolic process	GO:006027	9.8	SGSH, NAGLU, FUCA1, GUSB, IDS, IDUA
24	neuromuscular process controlling balance	GO:050885	9.8	GAA, HEXB, HEXA, TPP1, GM2A, CLN8
25	adult walking behavior	GO:007628	9.7	CLN8, HEXA, NPC1
26	glycosaminoglycan metabolic process	GO:030203	8.9	HEXB, HGSNAT, GALNS, HEXA, GLB1, GNS
27	sphingolipid metabolic process	GO:006665	8.9	GBA, GALC, NEU1, PSAP, HEXB, ARSH
28	glycosphingolipid metabolic process	GO:006687	8.9	ARSH, ARSA, ARSB, GM2A, GLA, GLB1
29	carbohydrate metabolic process	GO:005975	8.8	GALC, GALNS, GBA, HGSNAT, NAGLU, GUSB
30	cell death	GO:008219	8.8	SMPD1, HEXA, HEXB, DNAJC5, GBA, CTSD
31	phospholipid metabolic process	GO:006644	8.4	CLN8, ARSH, ARSA, GALC, GBA, ARSB
32	small molecule metabolic process	GO:044281	7.4	SGSH, SMPD1, HEXA, HEXB, PSAP, SLC44A4

Molecular functions related to lysosomal storage disease according to GeneDecks:

(show all 7)

id	Name	GO ID	Score	Top Affiliating Genes
1	galactoside binding	GO:016936	10.1	GLA, GLB1
2	sulfuric ester hydrolase activity	GO:008484	10.0	GALNS, GNS, ARSA, SGSH
3	arylsulfatase activity	GO:004065	10.0	ARSH, ARSA, ARSB
4	beta-N-acetylhexosaminidase activity	GO:004563	10.0	HEXB, HEXA, GM2A
5	N-acetylgalactosamine-4-sulfatase activity	GO:003943	9.9	ARSB, GALNS
6	mannose binding	GO:005537	9.8	CLN5, MRC1, MANBA, M6PR
7	cation binding	GO:043169	9.3	NAGA, GALC, GBA, HEXB, HEXA, GLB1