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P1134

Sigma-Aldrich

Porphobilinogen

powder

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Synonym(s):
5-(Aminomethyl)-4-(carboxymethyl)-1H-pyrrole-3-propanoic acid
Empirical Formula (Hill Notation):
C10H14N2O4
CAS Number:
487-90-1
Molecular Weight:
226.23
Beilstein:
220051
EC Number:
207-666-3
MDL number:
MFCD00005224
UNSPSC Code:
12352204
PubChem Substance ID:
24898159
NACRES:
NA.83

Assay

≥96% (HPLC)

Quality Level

200

form

powder

solubility

1 M NH4OH: 10 mg/mL

storage temp.

−20°C

SMILES string

NCc1[nH]cc(CCC(O)=O)c1CC(O)=O

InChI

1S/C10H14N2O4/c11-4-8-7(3-10(15)16)6(5-12-8)1-2-9(13)14/h5,12H,1-4,11H2,(H,13,14)(H,15,16)

InChI key

QSHWIQZFGQKFMA-UHFFFAOYSA-N

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Biochem/physiol Actions

Intermediate in the biosynthesis of heme.

Preparation Note

Enzymatically prepared.

Pictograms

Exclamation mark
GHS07

Signal Word

Warning

Hazard Statements

H315,H319,H335

Hazard Classifications

Eye Irrit. 2 - Skin Irrit. 2 - STOT SE 3

Target Organs

Respiratory system

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US), Eyeshields, Gloves

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The porphyrias are a group of rare, mainly inherited, diseases caused by a deficiency of one of the enzymes of the haem biosynthesis pathway. The biochemical hallmark of an acute attack is an increase in urine porphobilinogen (PBG), together with
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Acute intermittent porphyria (AIP) results from haplo-insufficiency of the porphobilinogen deaminase (PBGD) gene encoding the third enzyme in the haem biosynthesis pathway. As liver is the main organ of pathology for AIP, emerging therapies that restore enzyme hepatic levels are
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