1. Gene
  2. PAM - peptidylglycine alpha-amidating monooxygenase Gene

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

PAM Protein Structure

Cu2_monooxygen

Cu2_monooxygen: Copper type II ascorbate-dependent monooxygenase, N-terminal domain (59 - 174)

Cu2_monoox_C

Cu2_monoox_C: Copper type II ascorbate-dependent monooxygenase, C-terminal domain (196 - 344)

NHL

NHL: NHL repeat (578 - 605)

NHL

NHL: NHL repeat (630 - 659)

NHL

NHL: NHL repeat (683 - 711)

NHL

NHL: NHL repeat (779 - 806)

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  • 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

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