Nesidioblastosis

An inherited autosomal recessive syndrome characterized by the disorganized formation of new islets in the PANCREAS and CONGENITAL HYPERINSULINISM. It is due to focal hyperplasia of pancreatic ISLET CELLS budding off from the ductal structures and forming new islets of Langerhans. Mutations in the islet cells involve the potassium channel gene KCNJ11 or the ATP-binding cassette transporter gene ABCC8, both on CHROMOSOME 11.

Synonym
Nesidioblastosis

Excerpt	Reference
"Nesidioblastosis is a rare, but well-recognized disorder of persistent hyperinsulinemic hypoglycemia in infancy, but adult-onset nesidioblastosis associated with hyperinsulinemic hypoglycemia, termed noninsulinoma pancreatogenous hypoglycemic syndrome (NIPHS), has been reported."	( Hirata, Y; Ishizawa, M; Nishida, K; Sugiyama, T; Takada, Y; Takanishi, K; Tsujino, M, 2005)
"Nesidioblastosis is a rare cause of non insulinoma pancreatogenous hypoglycemic syndrome seen in adults."	( Gupta, RA; Nagral, S; Patel, RP, 2013)
"Nesidioblastosis is a term used to describe pathologic overgrowth of pancreatic islet cells."	( Bang, S; Choi, H; Kang, CM; Kang, H; Kim, HG; Kim, S; Lee, HW; Lim, TS, 2014)
"Although nesidioblastosis is a rare but well-recognized disorder of persistent hypoglycaemia in infants, it is extremely rare in adults."	( Blandamura, S; Merigliano, S; Pastorelli, D; Sperti, C; Valli, V, 2015)
"Nesidioblastosis is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults."	( Forrer, F; Horvath, K; Kump, P; Lackner, C; Mader, JK; Perren, A; Pieber, TR; Schwetz, V; Sourij, H; Treiber, G, 2016)
"Nesidioblastosis is a rare cause of adult hypoglycemia."	( Bonora, E; Castelli, C; Dauriz, M; Ferdeghini, M; Fuini, A; Landoni, L; Malleo, G; Maneschi, C; Moghetti, P; Tomezzoli, A, 2019)

Timeframe	Studies, This Condition (%)	All Conditions %
pre-1990	1 (3.33)	23.3326
1990's	0 (0.00)	12.5806
2000's	11 (36.67)	18.1394
2010's	17 (56.67)	28.8240
2020's	1 (3.33)	9.53

Drug	Relationship Strength	Studies
dihydroxyphenylalanine	low	1
amlodipine	low	1
diazoxide	low	8
nifedipine	low	1
calcium gluconate	low	3
fluorodopa f 18	low	1
fluorodeoxyglucose f18	low	1
glycogen	low	1
pasireotide	low	2
glucagon-like peptide 1	low	4
c-peptide	low	3

Protein Targets (250)

Protein	Potency Measurements	Inhibition Measurements	Activation Measurements	Drugs
Chain A, Ferritin light chain	3	0	0	3
acid sphingomyelinase	1	0	0	1
TDP1 protein	4	0	0	4
Smad3	2	0	0	2
cytochrome P450 family 3 subfamily A polypeptide 4	3	0	0	3
G	2	0	0	2
cytochrome P450 2D6	2	0	0	2
IDH1	2	0	0	2
chromobox protein homolog 1	2	0	0	2
NEDD8-activating enzyme E1 regulatory subunit isoform a	0	1	0	1
NEDD8-conjugating enzyme Ubc12	0	1	0	1
nuclear receptor ROR-gamma isoform 1	3	0	0	3
geminin	4	0	0	4
histone acetyltransferase KAT2A isoform 1	4	0	0	4
ATP-binding cassette sub-family C member 3	0	4	0	4
Multidrug resistance-associated protein 4	0	4	0	4
Voltage-dependent L-type calcium channel subunit alpha-1C	0	2	0	2
Potassium channel subfamily K member 2	0	1	2	3
Bile salt export pump	0	4	0	4
Interferon beta	2	0	0	2
HLA class I histocompatibility antigen, B alpha chain	2	0	0	2
ATP-dependent translocase ABCB1	0	2	0	2
Alpha-2A adrenergic receptor	0	2	0	2
Alpha-2C adrenergic receptor	0	2	0	2
Alpha-2B adrenergic receptor	0	1	0	1
Voltage-dependent L-type calcium channel subunit alpha-1C	0	3	0	3
Alpha-2C adrenergic receptor	0	1	0	1
Alpha-2A adrenergic receptor	0	1	0	1
Alpha-1D adrenergic receptor	0	1	0	1
Alpha-1A adrenergic receptor	0	1	0	1
Alpha-1B adrenergic receptor	0	1	0	1
5-hydroxytryptamine receptor 6	0	2	0	2
Spike glycoprotein	2	0	0	2
Sodium-dependent dopamine transporter	0	2	0	2
Voltage-dependent L-type calcium channel subunit alpha-1C	0	3	0	4
Potassium channel subfamily K member 2	0	2	0	2
Inositol hexakisphosphate kinase 1	2	0	0	2
Canalicular multispecific organic anion transporter 1	0	4	0	4
cytochrome P450 2C9, partial	2	0	0	2
Chain A, Beta-lactamase	2	0	0	2
ATAD5 protein, partial	2	0	0	2
GLS protein	2	0	0	2
aldehyde dehydrogenase 1 family, member A1	3	0	0	3
thyroid stimulating hormone receptor	2	0	0	2
regulator of G-protein signaling 4	2	0	0	2
estrogen nuclear receptor alpha	3	0	0	3
euchromatic histone-lysine N-methyltransferase 2	2	0	0	2
cytochrome P450 2D6 isoform 1	2	0	0	2
D(1A) dopamine receptor	2	0	0	2
thyroid hormone receptor beta isoform a	1	0	0	1
importin subunit beta-1 isoform 1	1	0	0	1
snurportin-1	1	0	0	1
GTP-binding nuclear protein Ran isoform 1	1	0	0	1
peripheral myelin protein 22	2	0	0	2
survival motor neuron protein isoform d	2	0	0	2
cytochrome P450 3A4 isoform 1	2	0	0	2
lamin isoform A-delta10	3	0	0	3
Gamma-aminobutyric acid receptor subunit pi	2	0	0	2
Coagulation factor VII	0	1	0	1
Tissue factor	0	1	0	1
Gamma-aminobutyric acid receptor subunit beta-1	2	0	0	2
Gamma-aminobutyric acid receptor subunit delta	2	0	0	2
Gamma-aminobutyric acid receptor subunit gamma-2	2	0	0	2
Glutamate receptor 1	1	0	0	3
Glutamate receptor 2	2	0	0	4
Glutamate receptor 3	1	0	0	3
Glutamate receptor 4	1	0	0	3
Gamma-aminobutyric acid receptor subunit alpha-5	2	0	0	2
Gamma-aminobutyric acid receptor subunit alpha-3	2	0	0	2
Gamma-aminobutyric acid receptor subunit gamma-1	2	0	0	2
Gamma-aminobutyric acid receptor subunit alpha-2	2	0	0	2
Gamma-aminobutyric acid receptor subunit alpha-4	2	0	0	2
Gamma-aminobutyric acid receptor subunit gamma-3	2	0	0	2
Gamma-aminobutyric acid receptor subunit alpha-6	2	0	0	2
Nicotinamide phosphoribosyltransferase	0	0	1	1
Gamma-aminobutyric acid receptor subunit alpha-1	2	0	0	2
Gamma-aminobutyric acid receptor subunit beta-3	2	0	0	2
Gamma-aminobutyric acid receptor subunit beta-2	2	0	0	2
ATP-binding cassette sub-family C member 8	0	1	1	2
ATP-sensitive inward rectifier potassium channel 11	0	1	1	2
GABA theta subunit	2	0	0	2
Gamma-aminobutyric acid receptor subunit epsilon	2	0	0	2
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE	2	0	0	2
Chain A, TYROSYL-DNA PHOSPHODIESTERASE	2	0	0	2
Chain A, Putative fructose-1,6-bisphosphate aldolase	2	0	0	2
Chain A, HADH2 protein	2	0	0	2
Chain B, HADH2 protein	2	0	0	2
Chain A, 2-oxoglutarate Oxygenase	2	0	0	2
Chain A, ATP-DEPENDENT DNA HELICASE Q1	1	0	0	1
endonuclease IV	1	0	0	1
15-lipoxygenase, partial	1	0	0	1
Microtubule-associated protein tau	2	0	0	2
Bloom syndrome protein isoform 1	2	0	0	2
polyunsaturated fatty acid lipoxygenase ALOX12	2	0	0	2
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 1	2	0	0	2
runt-related transcription factor 1 isoform AML1b	1	0	0	1
core-binding factor subunit beta isoform 2	1	0	0	1
mitogen-activated protein kinase 1	2	0	0	2
DNA polymerase kappa isoform 1	1	0	0	1
Epidermal growth factor receptor	0	1	0	1
Caspase-7	1	0	0	1
Glucagon-like peptide 1 receptor	0	0	1	1
Glucagon-like peptide 1 receptor	0	1	1	2
Glucagon receptor	0	0	1	1
Glucagon receptor	0	0	1	1
EWS/FLI fusion protein	1	0	0	1
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a	2	0	0	2
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a	2	0	0	2
Solute carrier family 22 member 2	0	1	0	1
Solute carrier family 22 member 1	0	1	0	1
Dihydrofolate reductase	0	1	0	1
Dipeptidyl peptidase 4	0	1	0	1
Solute carrier family 22 member 1	0	0	0	1
Multidrug and toxin extrusion protein 1	0	1	0	1
Chain A, JmjC domain-containing histone demethylation protein 3A	1	0	0	1
Luciferase	1	0	0	1
thioredoxin reductase	1	0	0	1
pregnane X receptor	1	0	0	1
phosphopantetheinyl transferase	1	0	0	1
hypoxia-inducible factor 1 alpha subunit	1	0	0	1
RAR-related orphan receptor gamma	1	0	0	1
Fumarate hydratase	1	0	0	1
USP1 protein, partial	1	0	0	1
NFKB1 protein, partial	1	0	0	1
GLI family zinc finger 3	1	0	0	1
Thrombopoietin	1	0	0	1
AR protein	1	0	0	1
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)	1	0	0	1
hypothetical protein, conserved	1	0	0	1
nuclear receptor subfamily 1, group I, member 3	1	0	0	1
progesterone receptor	1	0	0	1
nonstructural protein 1	1	0	0	1
glucocorticoid receptor [Homo sapiens]	1	0	0	1
retinoic acid nuclear receptor alpha variant 1	1	0	0	1
retinoid X nuclear receptor alpha	1	0	0	1
estrogen-related nuclear receptor alpha	1	0	0	1
farnesoid X nuclear receptor	1	0	0	1
pregnane X nuclear receptor	1	0	0	1
polyprotein	1	0	0	1
67.9K protein	1	0	0	1
Parkin	1	0	0	1
bromodomain adjacent to zinc finger domain 2B	1	0	0	1
peroxisome proliferator-activated receptor delta	1	0	0	1
peroxisome proliferator activated receptor gamma	1	0	0	1
vitamin D (1,25- dihydroxyvitamin D3) receptor	1	0	0	1
heat shock 70kDa protein 5 (glucose-regulated protein, 78kDa)	1	0	0	1
aryl hydrocarbon receptor	1	0	0	1
thyroid stimulating hormone receptor	1	0	0	1
v-jun sarcoma virus 17 oncogene homolog (avian)	1	0	0	1
beta-2 adrenergic receptor	1	0	0	1
peripheral myelin protein 22 isoform 1	1	0	0	1
cellular tumor antigen p53 isoform a	1	0	0	1
tumor necrosis factor	1	0	0	1
cytochrome P450 2C19 precursor	1	0	0	1
cytochrome P450 2C9 precursor	1	0	0	1
perilipin-5	0	1	0	1
vitamin D3 receptor isoform VDRA	1	0	0	1
perilipin-1	0	1	0	1
thyroid hormone receptor beta isoform 2	1	0	0	1
heat shock protein beta-1	1	0	0	1
atrial natriuretic peptide receptor 2 precursor	1	0	0	1
serine/threonine-protein kinase mTOR isoform 1	1	0	0	1
serine/threonine-protein kinase PLK1	1	0	0	1
eyes absent homolog 2 isoform a	1	0	0	1
histone-lysine N-methyltransferase 2A isoform 2 precursor	1	0	0	1
nuclear factor erythroid 2-related factor 2 isoform 1	1	0	0	1
DNA polymerase iota isoform a (long)	1	0	0	1
urokinase-type plasminogen activator precursor	1	0	0	1
plasminogen precursor	1	0	0	1
urokinase plasminogen activator surface receptor precursor	1	0	0	1
1-acylglycerol-3-phosphate O-acyltransferase ABHD5 isoform a	0	1	0	1
M-phase phosphoprotein 8	1	0	0	1
muscleblind-like protein 1 isoform 1	1	0	0	1
muscarinic acetylcholine receptor M1	1	0	0	1
Polyunsaturated fatty acid lipoxygenase ALOX15B	1	0	0	1
Voltage-dependent L-type calcium channel subunit alpha-1F	0	2	0	2
Bile salt export pump	0	1	0	1
5-hydroxytryptamine receptor 4	0	2	0	2
Voltage-dependent calcium channel gamma-2 subunit	1	0	0	1
Glutathione reductase, mitochondrial	0	2	0	2
Cellular tumor antigen p53	1	0	0	1
Integrin beta-3	1	0	0	1
Cytochrome P450 1A2	0	1	0	1
Muscarinic acetylcholine receptor M2	0	0	0	1
Muscarinic acetylcholine receptor M4	0	0	0	1
Integrin alpha-IIb	1	0	0	1
Cytochrome P450 3A4	0	1	0	1
Muscarinic acetylcholine receptor M5	0	0	0	1
Adenosine receptor A3	0	2	0	2
Muscarinic acetylcholine receptor M1	0	0	0	1
Cytochrome P450 2C9	0	1	0	1
Polyunsaturated fatty acid lipoxygenase ALOX15	0	1	0	1
Phospholipase A2, membrane associated	0	0	0	1
5-hydroxytryptamine receptor 2A	0	2	0	2
Alpha-1B adrenergic receptor	0	2	0	2
Muscarinic acetylcholine receptor M3	0	0	0	1
Potassium voltage-gated channel subfamily A member 5	0	1	0	1
3-oxo-5-alpha-steroid 4-dehydrogenase 1	0	1	0	1
Thromboxane-A synthase	0	1	0	1
Platelet-activating factor receptor	0	0	0	1
Voltage-dependent L-type calcium channel subunit alpha-1D	0	1	0	1
5-hydroxytryptamine receptor 2C	0	2	0	2
Indoleamine 2,3-dioxygenase 1	0	1	0	1
Adenosine receptor A2a	0	1	0	1
Adenosine receptor A1	0	2	0	2
Adenosine receptor A2a	0	1	0	1
3-oxo-5-alpha-steroid 4-dehydrogenase 2	0	1	0	1
Sodium channel protein type 1 subunit alpha	0	1	0	1
Sodium channel protein type 4 subunit alpha	0	1	0	1
C-C chemokine receptor type 2	0	2	0	2
Alpha-1A adrenergic receptor	0	2	0	2
Histamine H2 receptor	1	0	0	1
Nuclear receptor ROR-gamma	1	0	0	1
Cytochrome P450 2J2	0	1	0	1
Inositol monophosphatase 1	1	0	0	1
Sodium channel protein type 7 subunit alpha	0	1	0	1
Voltage-dependent L-type calcium channel subunit alpha-1D	0	2	0	2
Voltage-dependent L-type calcium channel subunit alpha-1C	0	1	0	1
Voltage-dependent L-type calcium channel subunit alpha-1S	0	1	0	1
Voltage-dependent L-type calcium channel subunit alpha-1S	0	1	0	1
Potassium voltage-gated channel subfamily A member 1	0	1	0	1
Potassium voltage-gated channel subfamily H member 2	0	1	0	1
C-terminal-binding protein 1	1	0	0	1
Voltage-dependent L-type calcium channel subunit alpha-1S	0	2	0	2
Calcium/calmodulin-dependent 3',5'-cyclic nucleotide phosphodiesterase 1C	0	1	0	1
Sodium channel protein type 5 subunit alpha	0	1	0	1
Leukotriene B4 receptor 1	0	0	0	1
Myosin light chain kinase, smooth muscle	0	1	0	1
Sodium channel protein type 9 subunit alpha	0	1	0	1
Transient receptor potential cation channel subfamily A member 1	0	0	1	1
Beta-2 adrenergic receptor	0	0	0	1
Indoleamine 2,3-dioxygenase 2	0	1	0	1
ATPase family AAA domain-containing protein 5	1	0	0	1
Voltage-dependent L-type calcium channel subunit alpha-1D	0	1	0	1
Sodium channel protein type 2 subunit alpha	0	1	0	1
Ataxin-2	1	0	0	1
Voltage-dependent L-type calcium channel subunit alpha-1F	0	1	0	1
Leukotriene B4 receptor 2	0	0	0	1
Sodium channel protein type 3 subunit alpha	0	1	0	1
Sodium channel protein type 11 subunit alpha	0	1	0	1
Broad substrate specificity ATP-binding cassette transporter ABCG2	0	1	0	1
Sodium channel protein type 8 subunit alpha	0	1	0	1
Sodium channel protein type 10 subunit alpha	0	1	0	1
ATP-dependent phosphofructokinase	1	0	0	1
interferon gamma precursor	0	0	0	1
Somatostatin receptor type 1	0	1	0	1
Somatostatin receptor type 2	0	1	0	1
Somatostatin receptor type 4	0	1	0	1
Somatostatin receptor type 3	0	1	0	1
Somatostatin receptor type 5	0	1	0	1
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

Synonyms(1)

Research Excerpts

Overview

Research

Studies (30)

Protein Targets (250)

Nesidioblastosis

Synonyms(1)

Research Excerpts

Overview

Research

Studies (30)

Drugs Studied (15)

Protein Targets (250)