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HMBS 抗体

HMBS 适用: 人, 小鼠, 大鼠 WB, IF (p), IHC (p) 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN1386078
发货至: 中国
  • 抗原 See all HMBS 抗体
    HMBS (Hydroxymethylbilane Synthase (HMBS))
    适用
    • 60
    • 35
    • 35
    • 5
    • 4
    • 4
    • 4
    • 3
    • 3
    • 2
    • 2
    • 2
    • 1
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    • 1
    人, 小鼠, 大鼠
    宿主
    • 54
    • 5
    • 1
    克隆类型
    • 48
    • 12
    多克隆
    标记
    • 25
    • 4
    • 3
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
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    This HMBS antibody is un-conjugated
    应用范围
    • 38
    • 12
    • 10
    • 8
    • 7
    • 5
    • 4
    • 4
    • 3
    • 1
    • 1
    • 1
    Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
    交叉反应
    人, 小鼠, 大鼠
    纯化方法
    Purified by Protein A.
    免疫原
    KLH conjugated synthetic peptide derived from human HMBS/PBGD
    亚型
    IgG
    Top Product
    Discover our top product HMBS Primary Antibody
  • 应用备注
    WB 1:300-5000
    IHC-P 1:200-400
    IF(IHC-P) 1:50-200
    限制
    仅限研究用
  • 状态
    Liquid
    浓度
    1 μg/μL
    缓冲液
    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
    储存液
    ProClin
    注意事项
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    储存条件
    4 °C,-20 °C
    储存方法
    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    有效期
    12 months
  • 抗原
    HMBS (Hydroxymethylbilane Synthase (HMBS))
    别名
    HMBS (HMBS 产品)
    别名
    PBG-D antibody, PBGD antibody, PORC antibody, UPS antibody, URO-S antibody, hemC antibody, pbg-d antibody, pbgd antibody, ups antibody, SPAC806.01 antibody, DDBDRAFT_0186148 antibody, DDBDRAFT_0231417 antibody, DDB_0186148 antibody, DDB_0231417 antibody, Hmbs antibody, NV50236 antibody, T25658 antibody, Ups antibody, Uros1 antibody, hmbs antibody, zgc:64128 antibody, hmbsl antibody, id:ibd5004 antibody, im:7140060 antibody, zgc:110690 antibody, hydroxymethylbilane synthase antibody, hydroxymethylbilane synthase S homeolog antibody, hydroxymethylbilane synthase L homeolog antibody, hydroxymethylbilane synthase (predicted) antibody, Hydroxymethylbilane synthase antibody, hydroxymethylbilane synthase a antibody, hydroxymethylbilane synthase, b antibody, HMBS antibody, Hmbs antibody, hmbs antibody, hmbs.2.S antibody, hmbs.L antibody, CNC02250 antibody, hem3 antibody, hemC antibody, Trad_0335 antibody, Plabr_2100 antibody, Sgly_3113 antibody, SMLT_RS19660 antibody, PMI_RS16580 antibody, hmbsa antibody, hmbsb antibody
    背景

    Synonyms: HEM3_HUMAN, HMBS, Hydroxymethylbilane synthase, PBG D, PBG-D, PBGD, Porphobilinogen deaminase, Pre uroporphyrinogen synthase, Pre-uroporphyrinogen synthase, UPS, Uroporphyrinogen I synthase, Uroporphyrinogen I synthetase.

    Background: PBGD, also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP). AIP is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.

    基因ID
    3145
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