catalog number :
MBS9329275
products type :
ELISA Kit
products full name :
Mouse Porphobilinogen deaminase, HMBS ELISA Kit
products short name :
Porphobilinogen deaminase, HMBS ELISA Kit
products name syn :
Hydroxymethylbilane synthase; Mouse Porphobilinogen deaminase (HMBS) ELISA kit; PBG-D; PBGD; UPS; porphobilinogen deaminase; pre-uroporphyrinogen synthase; uroporphyrinogen I synthase; uroporphyrinogen I synthetase; hydroxymethylbilane synthase
other names :
porphobilinogen deaminase isoform 1; Porphobilinogen deaminase; porphobilinogen deaminase; PBG-D; pre-uroporphyrinogen synthase; hydroxymethylbilane synthase; Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthase
products gene name :
HMBS
other gene names :
Hmbs; Hmbs; Ups; PBGD; Uros1; T25658; Uros1; PBG-D; HMBS
uniprot entry name :
HEM3_MOUSE
specificity :
No significant cross-reactivity or interference between this analyte and analogues is observed.
storage stability :
Store all reagents at 2-8 degree C
other info1 :
Samples: Undiluted original Mouse body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances. Assay Type: Sandwich. Detection Range: 0.625 ng/ml - 20 ng/ml. Sensitivity: 0.1 ng/ml.
other info2 :
Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].
products description :
Background/Introduction: This Quantitative Sandwich ELISA kit is only for in vitro research use only, NOT for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of HMBS (hereafter termed "analyte") in undiluted original Mouse body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.
ncbi acc num :
NP_038579.2
ncbi gb acc num :
NM_013551.2
ncbi mol weight :
39,344 Da
ncbi pathways :
Heme Biosynthesis Pathway (198305); Heme Biosynthesis Pathway (574886); Metabolism Pathway (574739); Metabolism Of Porphyrins Pathway (574885); Porphyrin And Chlorophyll Metabolism Pathway (83219); Porphyrin And Chlorophyll Metabolism Pathway (407); Heme Biosynthesis II Pathway (542887); Tetrapyrrole Biosynthesis II Pathway (142880)
uniprot summary :
HMBS: Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps. Defects in HMBS are the cause of acute intermittent porphyria (AIP). AIP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Belongs to the HMBS family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Mitochondrial; EC 2.5.1.61; Transferase; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll. Cellular Component: axon; cytoplasm; condensed chromosome; nucleus. Molecular Function: transferase activity; amine binding; carboxylic acid binding; uroporphyrinogen-III synthase activity; coenzyme binding; hydroxymethylbilane synthase activity. Biological Process: tetrapyrrole biosynthetic process; peptidyl-pyrromethane cofactor linkage; porphyrin biosynthetic process; heme biosynthetic process