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

PAM - peptidylglycine alpha-amidating monooxygenase Gene

  • 基因
  • 蛋白
  • 疾病
  • 相关产品
  • 直系同源

中文名称：肽基甘氨酸α-酰胺化单加氧酶

种属: Homo sapiens

同用名: PAL; PHM

基因 ID: 5066 | 基因类型: protein coding

关于 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.

功能概要

该基因编码一种多功能蛋白质。编码的前原蛋白经过蛋白水解处理以产生成熟酶。该酶包括两个具有不同催化活性的结构域，一个肽基甘氨酸 α-羟基化单加氧酶 (PHM) 结构域和一个肽基-α-羟基甘氨酸 α- 酰胺化裂解酶 (PAL) 结构域。这些催化结构域依次起作用以催化神经内分泌肽向活性α-酰胺化产物的转化。可变剪接导致多个转录本变体，其中至少一个编码一种经过蛋白水解处理的亚型。[RefSeq 提供，2016 年 1 月]

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 基因产物(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 蛋白结构

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)

  • 0
  • 200
  • 400
  • 600
  • 800
  • 973 a.a.
蛋白主名 其他名称

peptidyl-glycine alpha-amidating monooxygenase

pancreatic peptidylglycine alpha-amidating monooxygenase

重组 PAM 蛋白

目录号 产品名 蛋白编号 纯度
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%

关联疾病

疾病名称 别名
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
疾病名称 别名
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

相关产品

Approval
生物活性分子 (3)
目录号 产品名 作用方式
纯度 是否罕见病
HY-17575 Melphalan ≥98.0% 否
HY-A0106 Levamisole 99.96% 是
HY-B1200 Pralidoxime chloride Activator 99.73% 否
Clinical Phase
生物活性分子 (6)
目录号 产品名 作用方式
纯度 Phase
HY-128770 LY3154207 Agonist 99.87% Phase 2
HY-108703A Foliglurax monohydrochloride Agonist 98.93% Phase 2
HY-111052 AZD7325 99.27% 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
生物活性分子 (136)
Cat. No. 产品名 作用方式
纯度 是否罕见病
HY-P1180A Pam3CSK4 TFA Agonist 99.66% 否
HY-108471 CU-CPT22 Inhibitor ≥99.0% 否
HY-110279 Ogerin Modulator 99.58% 否
HY-112146 MMG-11 Antagonist 99.11% 否
HY-115467 MitoBloCK-10 98.89% 否
HY-113320 Etiocholanolone Modulator 99.31% 否
HY-120023 VU0453595 Agonist 99.90% 否
HY-120874 PF-06372865 Activator 99.29% 否
HY-120613 BMS-986187 Activator 98.93% 否
HY-143312 V-0219 Modulator 98.65% 否
HY-12158 VU0238441 Activator 99.88% 否
HY-124419 RO0711401 Agonist 98.42% 否
HY-114933 VU0119498 Modulator 99.52% 否
HY-129086 BPAM344 Agonist 99.68% 否
HY-18654 ADX88178 Agonist 99.69% 否
HY-12439 ML380 Agonist 99.91% 否
HY-14418 VU0361737 Agonist 99.79% 否
HY-100750 Norverapamil hydrochloride Inhibitor 99.80% 否
HY-116819A VU0453379 hydrochloride Modulator ≥99.0% 否
HY-141899 MK-6884 Modulator 99.11% 否
HY-143312A V-0219 hydrochloride Modulator 99.65% 否
HY-131004 CB2R PAM Agonist 98.46% 否
HY-P1181A Pam2CSK4 TFA Agonist 99.28% 否
HY-13058 ADX-47273 Agonist 99.94% 否
HY-100605 VU0483605 Modulator 97.03% 否
HY-113346 Tetrahydrodeoxycorticosterone Agonist 99.45% 否
HY-136190 TRPC6-PAM-C20 Agonist 99.90% 否
HY-14417 VU0155041 Agonist 99.10% 否
HY-100588 VU0364770 Agonist 99.93% 否
HY-120184 VU0467485 Agonist ≥99.0% 否
HY-122255 LY487379 Inhibitor 98.88% 否
HY-148867 UCM-1306 Modulator 99.61% 否
HY-15476 VU 0364439 Agonist 98.36% 否
HY-112146A MMG-11 quarterhydrate Antagonist 99.09% 否
HY-113689 GAT211 Agonist 99.98% 否
HY-116819 VU0453379 Modulator ≥98.0% 否
HY-126327A UNC4976 TFA Antagonist ≥98.0% 否
HY-143312E (S)-V-0219 hydrochloride Modulator 99.71% 否
HY-162455 NA-014 Agonist 99.49% 否
HY-14612 CPPHA Modulator 98.04% 否
HY-103475 GS39783 Agonist 99.72% 否
HY-113320S Etiocholanolone-d5 Modulator 99.20% 否
HY-116463 E1R Agonist 99.83% 否
HY-119078 VU0080241 Modulator 99.69% 否
HY-120530 JNJ-46281222 Inhibitor 98.77% 否
HY-122819 Calindol hydrochloride Agonist 99.88% 否
HY-126327 UNC4976 Antagonist / 否
HY-137204 COR659 Activator 99.94% 否
HY-141515 BMS-986121 Modulator 98.89% 否
HY-143312D (R)-V-0219 hydrochloride Modulator 99.37% 否
HY-W115727A Polyacrylamide,Anion,Mw 14-16 million / 否
HY-12149 A-867744 Agonist 99.76% 否
HY-114863 PHCCC(4Me) Modulator 99.68% 否
HY-116855 TASP0433864 Modulator 99.9% 否
HY-117851 AC-265347 Agonist 99.97% 否
HY-119226 VU0152099 Agonist 98.25% 否
HY-124821 VA012 Modulator / 否
HY-139044 VU6000918 Activator 99.36% 否
HY-14419 TCN238 Modulator 99.57% 否
HY-149453 MCUF-651 Modulator 99.85% 否
HY-16951 VU-1545 Agonist 99.00% 否
HY-P1180 Pam3CSK4 Agonist / 否
HY-11048 NS11394 Modulator 99.77% 否
HY-113346S Tetrahydrodeoxycorticosterone-d3 Agonist 98.50% 否
HY-15393 VU 0357121 Agonist 99.73% 否
HY-W338852 Gentisuric acid 99.65% 否
HY-112814 VU6001376 / 否
HY-128584 VU6005806 Agonist / 否
HY-101841 LY 2033298 Activator / 否
HY-103552 LY487379 hydrochloride Modulator / 否
HY-103571 VU0285683 Antagonist / 否
HY-107423 GNE 6901 Agonist / 否
HY-107504 VU0360172 hydrochloride Agonist / 否
HY-107506 Ro 67-4853 Agonist 99.90% 否
HY-113320S1 Etiocholanolone-d2 Modulator / 否
HY-113608 VCP171 Modulator / 否
HY-114978 VU0424465 Agonist / 否
HY-116463A (2R,3S)-E1R Agonist 98.84% 否
HY-116463B (2S,3S)-E1R Agonist 98.24% 否
HY-116463C (2R,3R)-E1R Agonist 98.79% 否
HY-116463D (Rac)-E1R Agonist 98.48% 否
HY-117408 VU6004256 Modulator / 否
HY-117611 GAT107 Modulator / 否
HY-118179 VU0486321 / 否
HY-118256 LSN2814617 Modulator / 否
HY-118416 BMS-986124 Antagonist / 否
HY-120004 PF-06827443 / 否
HY-120576 ML169 Modulator / 否
HY-120727 VU0364289 Activator 99.52% 否
HY-121806 VU0486846 Modulator / 否
HY-124112 PAM 1392 Inhibitor / 否
HY-124372 JNJ-46356479 Modulator / 否
HY-128783 VU0090157 Modulator / 否
HY-136258 nAChR agonist CMPI hydrochloride Agonist / 否
HY-143312B (R)-V-0219 Modulator / 否
HY-143312C (S)-V-0219 Modulator / 否
HY-14417B VU0155041 sodium Agonist / 否
HY-147421 Xilmenolone Modulator / 否
HY-149975 AMPA receptor modulator-4 Activator / 否
HY-151899 A3AR modulator 1 Agonist / 否
HY-155628 AMPA receptor modulator-6 Modulator / 否
HY-156331 VU6004909 Activator 99.40% 否
HY-157421 Nampt activator-4 Modulator / 否
HY-159177 M4 mAChR Modulator-1 Modulator / 否
HY-159509 Claziprotamidum / 否
HY-159624 KK-92A Modulator / 否
HY-160529 α7 nAChR Modulator-2 Modulator / 否
HY-161810 MRS8247 Modulator / 否
HY-162454 DA-023 Modulator / 否
HY-162662 VU6008677 / 否
HY-163280 JGB-1-155 Activator / 否
HY-168025 VU6007496 / 否
HY-169247 Pam2CS-DMAPA / 否
HY-18679 TC-N 22A Agonist / 否
HY-19623 VU0092273 Agonist / 否
HY-P1180B Pam3CSK4 trihydrochloride / 否
HY-P1181 Pam2CSK4 Agonist / 否
HY-P1405 Pam3CSK4-Biotin Agonist / 否
HY-RS10011 PAM Human Pre-designed siRNA Set A Pre-designed Sets / 否
HY-RS10012 PAM16 Human Pre-designed siRNA Set A Pre-designed Sets / 否
HY-W115727 Polyacrylamide,Anion,Mw 10-12 million / 否
HY-W115727B Polyacrylamide,Anion,Mw 18 million / 否
HY-103535 GBLD345 Agonist / 否
HY-116149 A-424274 / 否
HY-118022 VU0361747 / 否
HY-119772 VU0366369 Modulator / 否
HY-122164 LY-503430 Agonist / 否
HY-123667 NCFP Modulator / 否
HY-123934 VU6007477 Modulator / 否
HY-129517 UBP714 Agonist / 否
HY-155467 MRS7935 Modulator / 否
HY-161113 Z8554052021 Activator / 否
HY-161118 MB327 / 否
HY-167922 (R,R,S)-GAT107 / 否
HY-W115727D Polyacrylamide,average Mn 40000 / 否
HY-W115727E Polyacrylamide,average Mn 150000 / 否

直系同源

种属 基因名 来源 基因 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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