PAM - peptidylglycine alpha-amidating monooxygenase Gene

Homo sapiens

Also known as PAL; PHM

Gene ID: 5066 | Gene type: protein coding

About PAM

Cytogenetic location: 5q21.1 Genomic coordinates (GRCh38): 5:102,754,783-103,031,105 (from NCBI)

This gene has 32 transcripts (splice variants) and 208 orthologues. Broad expression in heart (RPKM 269.6), adrenal (RPKM 54.3) and 15 other tissues.

Summary

This gene encodes a multifunctional protein. The encoded preproprotein is proteolytically processed to generate the mature Enzyme. This Enzyme includes two domains with distinct catalytic activities, a peptidylglycine alpha-hydroxylating monooxygenase (PHM) domain and a peptidyl-alpha-hydroxyglycine alpha-amidating lyase (PAL) domain. These catalytic domains work sequentially to catalyze the conversion of neuroendocrine Peptides to active alpha-amidated products. Alternative splicing results in multiple transcript variants, at least one of which encodes an isoform that is proteolytically processed. [provided by RefSeq, Jan 2016]

PAM Products(19)

mRNA	Protein	Name
NM_000919.4	NP_000910.2	peptidyl-glycine alpha-amidating monooxygenase isoform a precursor
NM_001177306.2	NP_001170777.1	peptidyl-glycine alpha-amidating monooxygenase isoform e preproprotein
NM_001319943.1	NP_001306872.1	peptidyl-glycine alpha-amidating monooxygenase isoform f precursor
NM_001364582.2	NP_001351511.1	peptidyl-glycine alpha-amidating monooxygenase isoform g precursor
NM_001364583.2	NP_001351512.1	peptidyl-glycine alpha-amidating monooxygenase isoform h precursor
NM_001364584.2	NP_001351513.1	peptidyl-glycine alpha-amidating monooxygenase isoform i precursor
NM_001364585.2	NP_001351514.1	peptidyl-glycine alpha-amidating monooxygenase isoform j precursor
NM_001364586.2	NP_001351515.1	peptidyl-glycine alpha-amidating monooxygenase isoform k precursor
NM_001364587.2	NP_001351516.1	peptidyl-glycine alpha-amidating monooxygenase isoform l precursor
NM_001364588.2	NP_001351517.1	peptidyl-glycine alpha-amidating monooxygenase isoform m precursor
NM_001364589.2	NP_001351518.1	peptidyl-glycine alpha-amidating monooxygenase isoform n precursor
NM_001364590.2	NP_001351519.1	peptidyl-glycine alpha-amidating monooxygenase isoform o
NM_001364591.2	NP_001351520.1	peptidyl-glycine alpha-amidating monooxygenase isoform p precursor
NM_001364592.2	NP_001351521.1	peptidyl-glycine alpha-amidating monooxygenase isoform q precursor
NM_001364593.2	NP_001351522.1	peptidyl-glycine alpha-amidating monooxygenase isoform r precursor
NM_001364594.2	NP_001351523.1	peptidyl-glycine alpha-amidating monooxygenase isoform s precursor
NM_138766.2	NP_620121.1	peptidyl-glycine alpha-amidating monooxygenase isoform b precursor
NM_138821.2	NP_620176.1	peptidyl-glycine alpha-amidating monooxygenase isoform c precursor
NM_138822.2	NP_620177.1	peptidyl-glycine alpha-amidating monooxygenase isoform d precursor

Gene Ontology

Molecular Function
Biological Process

Molecular Function GO Annotation	Evidence	Reference	Source
enables peptidylamidoglycolate lyase activity	IDA IDA: Inferred from direct assay	12699694	GOA
enables peptidylglycine monooxygenase activity	IDA IDA: Inferred from direct assay	2357221	GOA
enables zinc ion binding	IDA IDA: Inferred from direct assay	12699694	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in peptide amidation	IDA IDA: Inferred from direct assay	2357221	GOA
involved in response to zinc ion	IDA IDA: Inferred from direct assay	12699694	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

PAM Protein Structure

Cu2_monooxygen

Cu2_monoox_C

NHL

0
200
400
600
800
973 a.a.

Protein Preferred Names

Protein Names

peptidyl-glycine alpha-amidating monooxygenase

pancreatic peptidylglycine alpha-amidating monooxygenase

Recombinant PAM Proteins

Cat. No.	Product Name	Accession	Purity
HY-P73731	PAM Protein, Human (HEK293, His)	P19021-2/NP_620176.1 (F21-S866)	≥95%
HY-P73732	PAM Protein, Human (HEK293, Fc)	P19021-2/NP_620176.1 (F21-V710)	≥95%

Related Diseases

Diseases

Alias

Menkes Disease

Copper Transport Disease	Menkes Syndrome
MNK	Kinky Hair Disease
Steely Hair Disease	Menkes Kinky-Hair Syndrome
Mk	Steely Hair Syndrome
Menkea Syndrome	Md
Menkes Kinky Hair Syndrome	Hypocupremia, Congenital
Kinky Hair Syndrome	X-Linked Copper Deficiency
Menkes Kinky Hair Disease

Spinal Muscular Atrophy, Distal, X-Linked 3

SMAX3	Dsmax
X-Linked Distal Spinal Muscular Atrophy Type 3	X-Linked Distal Spinal Muscular Atrophy 3
Atp7a-Related Distal Motor Neuropathy	X-Linked Dhmn3
X-Linked Distal Hereditary Motor Neuropathy Type 3	X-Linked Dsma3
Spinal Muscular Atrophy, Distal, X-Linked Recessive	X-Linked Recessive Distal Spinal Muscular Atrophy
Distal Spinal Muscular Atrophy, X-Linked, 3	DSMAX3
Spinal Muscular Atrophy Distal X-Linked Recessive	Atrophy, Muscular, Spinal, Distal, X-Linked, Type 3

Schizophrenia 1

SCZD1	Schizophrenia Susceptibility Locus, Chromosome 5-Related
Schizophrenia 1 With Or Without An Affective Disorder

Copper Deficiency Myelopathy

Swayback

Human Swayback

Occipital Horn Syndrome

OHS	Eds Ix
Cutis Laxa X-Linked	Cutis Laxa, X-Linked
Cutis Laxa, X-Linked, Formerly	Ehlers-Danlos Syndrome, Occipital Horn Type, Formerly
Eds Ix, Formerly	Eds9, Formerly
Ehlers-Danlos Syndrome Type 9	Ehlers-Danlos Syndrome Type Ix
X-Linked Cutis Laxa	Ehlers-Danlos Syndrome, Occipital Horn Type
Eds9	Ehlers-Danlos Syndrome Occipital Horn Type

Postpoliomyelitis Syndrome

Postpolio Syndrome	Post-Polio Syndrome
Post Polio Syndrome	Polio Late Effects
Post-Polio Muscular Atrophy	Post-Polio Sequelae
Post-Poliomyelitic Syndrome	Postpolio Sequelae
Postpoliomyelitic Syndrome	Postpoliomyelitis Sequelae

Mental Retardation, Enteropathy, Deafness, Peripheral Neuropathy, Ichthyosis, And Keratoderma

Mednik Syndrome	Erythrokeratodermia Variabilis 3
MEDNIK	Ekv3
Erythrokeratodermia Variabilis, Kamouraska Type	Mental Retardation, Enteropathy, Deafness, Neuropathy, Ichthyosis, Keratodermia
Intellectual Disability-Enteropathy-Deafness-Peripheral Neuropathy-Ichthyosis-Keratodermia Syndrome	Intellectual Disability-Enteropathy-Hearing Loss-Peripheral Neuropathy-Ichthyosis-Keratodermia Syndrome
Intellectual Disability, Enteropathy, Deafness, Peripheral Neuropathy, Ichthyosis, And Keratoderma	Erythrokeratodermia Variabilis Kamouraska Type
Mental Retardation, Enteropathy, Deafness, Peripheral Neuropathy, Ichthyosis, Keratoderma

Hypoascorbemia

Scurvy	Ascorbic Acid Deficiency
Vitamin C Deficiency	Vitamin C, Inability To Synthesize
Deficiency Of Vitamin C	Scorbutus
Vitamin C

Aceruloplasminemia

Cerebellar Ataxia	Hypoceruloplasminemia
Hemosiderosis, Systemic, Due To Aceruloplasminemia	Familial Apoceruloplasmin Deficiency
Hereditary Ceruloplasmin Deficiency	Deficiency Of Ferroxidase
Hypoceruloplasminemia, Hereditary	Ceruloplasmin Deficiency
Systemic Hemosiderosis Due To Aceruloplasminemia	ACERULOP

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Approval

Bioactive Molecules (2)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-A0106	Levamisole		99.96%	Yes
HY-B1200	Pralidoxime chloride	Activator	99.73%	Unkown

Clinical Phase

Bioactive Molecules (4)

Cat. No.	Product Name	Effect	Purity	Phase
HY-128770	LY3154207	Agonist	99.87%	Phase 2
HY-132806	Alogabat	Activator	99.93%	Phase 2
HY-108703	Foliglurax	Agonist	/	Phase 2
HY-144291	LY3154885	Agonist	/	Phase 1

Pre-clinical Phase

Bioactive Molecules (137)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-P1180A	Pam3CSK4 TFA	Agonist	99.66%	Unkown
HY-108471	CU-CPT22	Inhibitor	≥99.0%	Unkown
HY-110279	Ogerin	Modulator	99.58%	Unkown
HY-112146	MMG-11	Antagonist	99.11%	Unkown
HY-115467	MitoBloCK-10		98.89%	Unkown
HY-113320	Etiocholanolone	Modulator	99.31%	Unkown
HY-120023	VU0453595	Agonist	99.90%	Unkown
HY-120874	PF-06372865	Activator	99.29%	Unkown
HY-108703A	Foliglurax monohydrochloride	Agonist	98.93%	Yes
HY-120613	BMS-986187	Activator	98.93%	Unkown
HY-143312	V-0219	Modulator	98.65%	Unkown
HY-12158	VU0238441	Activator	99.88%	Unkown
HY-124419	RO0711401	Agonist	98.42%	Unkown
HY-111052	AZD7325		99.27%	Unkown
HY-114933	VU0119498	Modulator	99.52%	Unkown
HY-18654	ADX88178	Agonist	99.69%	Unkown
HY-129086	BPAM344	Agonist	99.68%	Unkown
HY-12439	ML380	Agonist	99.91%	Unkown
HY-14418	VU0361737	Agonist	99.79%	Unkown
HY-116819A	VU0453379 hydrochloride	Modulator	≥99.0%	Unkown
HY-131004	CB2R PAM	Agonist	98.46%	Unkown
HY-141899	MK-6884	Modulator	99.11%	Unkown
HY-143312A	V-0219 hydrochloride	Modulator	99.65%	Unkown
HY-13058	ADX-47273	Agonist	99.94%	Unkown
HY-P1181A	Pam2CSK4 TFA	Agonist	99.28%	Unkown
HY-100605	VU0483605	Modulator	97.03%	Unkown
HY-113346	Tetrahydrodeoxycorticosterone	Agonist	99.45%	Unkown
HY-136190	TRPC6-PAM-C20	Agonist	99.90%	Unkown
HY-14417	VU0155041	Agonist	99.10%	Unkown
HY-100588	VU0364770	Agonist	99.93%	Unkown
HY-120184	VU0467485	Agonist	≥99.0%	Unkown
HY-122255	LY487379	Inhibitor	98.88%	Unkown
HY-148867	UCM-1306	Modulator	99.61%	Unkown
HY-15476	VU 0364439	Agonist	98.36%	Unkown
HY-112146A	MMG-11 quarterhydrate	Antagonist	99.09%	Unkown
HY-113689	GAT211	Agonist	99.98%	Unkown
HY-116819	VU0453379	Modulator	≥98.0%	Unkown
HY-126327A	UNC4976 TFA	Antagonist	≥98.0%	Unkown
HY-143312E	(S)-V-0219 hydrochloride	Modulator	99.71%	Unkown
HY-162455	NA-014	Agonist	99.49%	Unkown
HY-14612	CPPHA	Modulator	98.04%	Unkown
HY-103475	GS39783	Agonist	99.72%	Unkown
HY-113320S	Etiocholanolone-d5	Modulator	99.20%	Unkown
HY-116463	E1R	Agonist	99.83%	Unkown
HY-119078	VU0080241	Modulator	99.69%	Unkown
HY-120530	JNJ-46281222	Inhibitor	98.77%	Unkown
HY-122819	Calindol hydrochloride	Agonist	99.88%	Unkown
HY-126327	UNC4976	Antagonist	/	Unkown
HY-137204	COR659	Activator	99.94%	Unkown
HY-141515	BMS-986121	Modulator	98.89%	Unkown
HY-143312D	(R)-V-0219 hydrochloride	Modulator	99.37%	Unkown
HY-W115727A	Polyacrylamide,Anion,Mw 14-16 million		/	Unkown
HY-12149	A-867744	Agonist	99.76%	Unkown
HY-114863	PHCCC(4Me)	Modulator	99.68%	Unkown
HY-116855	TASP0433864	Modulator	99.9%	Unkown
HY-117851	AC-265347	Agonist	99.97%	Unkown
HY-119226	VU0152099	Agonist	98.25%	Unkown
HY-124821	VA012	Modulator	/	Unkown
HY-139044	VU6000918	Activator	99.36%	Unkown
HY-14419	TCN238	Modulator	99.57%	Unkown
HY-149453	MCUF-651	Modulator	99.85%	Unkown
HY-16951	VU-1545	Agonist	99.00%	Unkown
HY-P1180	Pam3CSK4	Agonist	/	Unkown
HY-11048	NS11394	Modulator	99.77%	Unkown
HY-113346S	Tetrahydrodeoxycorticosterone-d3	Agonist	98.50%	Unkown
HY-15393	VU 0357121	Agonist	99.73%	Unkown
HY-W338852	Gentisuric acid		99.65%	Unkown
HY-112814	VU6001376		/	Unkown
HY-128584	VU6005806	Agonist	/	Unkown
HY-101841	LY 2033298	Activator	/	Unkown
HY-103552	LY487379 hydrochloride	Modulator	/	Unkown
HY-103571	VU0285683	Antagonist	/	Unkown
HY-107423	GNE 6901	Agonist	/	Unkown
HY-107504	VU0360172 hydrochloride	Agonist	/	Unkown
HY-107506	Ro 67-4853	Agonist	99.90%	Unkown
HY-113320S1	Etiocholanolone-d2	Modulator	/	Unkown
HY-113608	VCP171	Modulator	/	Unkown
HY-114978	VU0424465	Agonist	/	Unkown
HY-116463A	(2R,3S)-E1R	Agonist	98.84%	Unkown
HY-116463B	(2S,3S)-E1R	Agonist	98.24%	Unkown
HY-116463C	(2R,3R)-E1R	Agonist	98.79%	Unkown
HY-116463D	(Rac)-E1R	Agonist	98.48%	Unkown
HY-117408	VU6004256	Modulator	/	Unkown
HY-117611	GAT107	Modulator	/	Unkown
HY-118179	VU0486321		/	Unkown
HY-118256	LSN2814617	Modulator	/	Unkown
HY-118416	BMS-986124	Antagonist	/	Unkown
HY-120004	PF-06827443		/	Unkown
HY-120576	ML169	Modulator	/	Unkown
HY-120727	VU0364289	Activator	99.52%	Unkown
HY-121806	VU0486846	Modulator	/	Unkown
HY-124112	PAM 1392	Inhibitor	/	Unkown
HY-124372	JNJ-46356479	Modulator	/	Unkown
HY-128783	VU0090157	Modulator	/	Unkown
HY-136258	nAChR agonist CMPI hydrochloride	Agonist	/	Unkown
HY-143312B	(R)-V-0219	Modulator	/	Unkown
HY-143312C	(S)-V-0219	Modulator	/	Unkown
HY-14417B	VU0155041 sodium	Agonist	/	Unkown
HY-147421	Xilmenolone	Modulator	/	Unkown
HY-149975	AMPA receptor modulator-4	Activator	/	Unkown
HY-151899	A3AR modulator 1	Agonist	/	Unkown
HY-155628	AMPA receptor modulator-6	Modulator	/	Unkown
HY-156331	VU6004909	Activator	99.40%	Unkown
HY-157421	Nampt activator-4	Modulator	/	Unkown
HY-159177	M4 mAChR Modulator-1	Modulator	/	Unkown
HY-159509	Claziprotamidum		/	Unkown
HY-159624	KK-92A	Modulator	/	Unkown
HY-160529	α7 nAChR Modulator-2	Modulator	/	Unkown
HY-161810	MRS8247	Modulator	/	Unkown
HY-162454	DA-023	Modulator	/	Unkown
HY-162662	VU6008677		/	Unkown
HY-163280	JGB-1-155	Activator	/	Unkown
HY-168025	VU6007496		/	Unkown
HY-169247	Pam2CS-DMAPA		/	Unkown
HY-18679	TC-N 22A	Agonist	/	Unkown
HY-19623	VU0092273	Agonist	/	Unkown
HY-P1180B	Pam3CSK4 trihydrochloride		/	Unkown
HY-P1181	Pam2CSK4	Agonist	/	Unkown
HY-P1405	Pam3CSK4-Biotin	Agonist	/	Unkown
HY-RS10011	PAM Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown
HY-RS10012	PAM16 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown
HY-W115727	Polyacrylamide,Anion,Mw 10-12 million		/	Unkown
HY-W115727B	Polyacrylamide,Anion,Mw 18 million		/	Unkown
HY-103535	GBLD345	Agonist	/	Unkown
HY-116149	A-424274		/	Unkown
HY-118022	VU0361747		/	Unkown
HY-119772	VU0366369	Modulator	/	Unkown
HY-122164	LY-503430	Agonist	/	Unkown
HY-123667	NCFP	Modulator	/	Unkown
HY-123934	VU6007477	Modulator	/	Unkown
HY-129517	UBP714	Agonist	/	Unkown
HY-155467	MRS7935	Modulator	/	Unkown
HY-161113	Z8554052021	Activator	/	Unkown
HY-161118	MB327		/	Unkown
HY-167922	(R,R,S)-GAT107		/	Unkown
HY-W115727D	Polyacrylamide,average Mn 40000		/	Unkown
HY-W115727E	Polyacrylamide,average Mn 150000		/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Rattus norvegicus	PAM	RGD	RGD:3252
Bos taurus	PAM	VGNC	VGNC:32561
Macaca mulatta	PAM	VGNC	VGNC:75752
Mus musculus	PAM	MGD	MGI:97475
Canis familiaris	PAM	VGNC	VGNC:44250
Felis catus	PAM	VGNC	VGNC:68690
Others	PAM	NCBI

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