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Product Details
14797-1-AP
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
HMBS Fusion Protein Ag6509 (1-361 aa encoded by BC000520)
Conjugate
Form
Liquid
Concentration
0.2 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Wet ice (domestic); Dry ice (international)
Product Specific Information
Immunogen sequence: MSGNGNAAA TAEENSPKMR VIRVGTRKSQ LARIQTDSVV ATLKASYPGL QFEIIAMSTT GDKILDTALS KIGEKSLFTK ELEHALEKNE VDLVVHSLKD LPTVLPPGFT IGAICKRENP HDAVVFHPKF VGKTLETLPE KSVVGTSSLR RAAQLQRKFP HLEFRSIRGN LNTRLRKLDE QQEFSAIILA TAGLQRMGWH NRVGQILHPE ECMYAVGQGA LGVEVRAKDQ DILDLVGVLH DPETLLRCIA ERAFLRHLEG GCSVPVAVHT AMKDGQLYLT GGVWSLDGSD SIQETMQATI HVPAQHEDGP EDDPQLVGIT ARNIPRGPQL AAQNLGISLA NLLLSKGAKN ILDVARQLND AH (1-361 aa encoded by BC000520)
Target Information
PBGD (porphobilinogen deaminase), 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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: alternative name: porphobilinogen deaminase; HEM3; HMBS; Hydroxymethylbilane synthase; PBG D; PBG-D; Porphobilinogen deaminase; porphyria, acute; Chester type; Pre-uroporphyrinogen synthase; uroporphyrinogen I synthase; uroporphyrinogen I synthetase
Gene Aliases: hemC; HMBS; PBG-D; PBGD; PORC; T25658; UPS; URO-S; Uros1
UniProt ID: (Human) P08397, (Mouse) P22907
Entrez Gene ID: (Human) 3145, (Mouse) 15288, (Rat) 25709
response to hypoxia
protoporphyrinogen IX biosynthetic process
heme biosynthetic process
response to carbohydrate
response to zinc ion
peptidyl-pyrromethane cofactor linkage
organ regeneration
response to cobalt ion
response to estradiol
response to vitamin
response to drug
response to amino acid
astrocyte differentiation
response to methylmercury
cellular response to antibiotic
cellular response to arsenic-containing substance
cellular response to lead ion
cellular response to cytokine stimulus
cellular response to amine stimulus
cellular response to dexamethasone stimulus
porphyrin-containing compound biosynthetic process
tetrapyrrole biosynthetic process
response to hormone
response to metal ion
response to lead ion
response to organic cyclic compound
response to nutrient levels
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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