Medical Isotopes, Inc. is a premier manufacturer of stable isotope chemicals labeled with: Deuterium, C13, N15, O18 and metal isotopes.  We specialize in custom synthesis with isotopes and non-labeled compounds. www.medicalisotopes.com
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Parent Category: Reference Standards
Reference Standards for Inborn Errors

Inborn errors of metabolism are inherited genetic disorders that affect one or more of the hundreds of biochemical pathways in the human body. Patients with these disorders are unable to properly use or synthesize certain compounds, such as fatty acids, amino acids, organic acids, or macromolecules, because of defects in the enzymes or other components of various metabolic pathways. These conditions frequently are identified in infants and young children with acute or chronic symptoms. When possible, early diagnoses with timely and effective interventions are essential for preventing permanent neurologic sequelae, disabilities, and other severe adverse outcomes. Biochemical genetic testing is a critical discipline in laboratory medicine for the evaluation, diagnosis, treatment monitoring, clinical management, and in some cases, carrier status assessment, of inherited metabolic diseases. These tests comprise highly complex and specialized laboratory procedures performed for evaluating enzyme activity, functional status of proteins, and levels of metabolites such as amino acids, organic acids, and fatty acids using a wide variety of specimen types including urine, whole blood, plasma, serum, cerebrospinal fluid, muscle biopsy, and other tissue types. Biochemical genetic tests also are among the critical follow-up procedures for diagnosing presumptive cases detected during newborn screening.

Catalog Number Product Name Enrichment

Quantity

Price

 

 
 
Acetyl carnitine
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Acetyl-d3-L-carnitine HCl
99% D
0.1 g $855     Add to Cart  
0.25 g $1,595     Add to Cart  
 
Acetyl-L-carnitine-d3 HCl (N-methyl-d3)
99%
50 mg $790     Add to Cart  
 
Acetyl-L-carnitine HCl
5 mg $495     Add to Cart  
100 mg $1,500     Add to Cart  
 
Acryloyl glycine
5 mg $790     Add to Cart  
 
Butyroyl carnitine
5 mg $820     Add to Cart  
 
Butyroyl glycine
5 mg $790     Add to Cart  
 
Butyroyl N-(methyl-d3)-carnitine
98%D
5 mg $1,200     Add to Cart  
 
n-Butyryl Carnitine Chloride
5 mg $950     Add to Cart  
 
Butyryl-L-carnitine-d3 HCl (N-methyl-d3)
99%D
0.01 g $780     Add to Cart  
0.05 g $1,695     Add to Cart  
 
DL-Carnitine-d9 HCl(trimethyl-d9)
98%D
10 mg $190     Add to Cart  
50 mg $590     Add to Cart  
 
L-Carnitine-d3 HCl (methyl-d3)
99%D
5 mg $390     Add to Cart  
 
L-Carnitine HCl
1 g $390     Add to Cart  
5 g $895     Add to Cart  
 
L-CARNITINE L-TARTRATE / BASE
5 mg Request Quote  
50 mg Request Quote  
 
Caspofungin acetate C0 analog
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Crotonyl glycine
5 mg $790     Add to Cart  
 
2,4-Decadienoic acid
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Decanoyl Carnitine Chloride
5 mg $1,200     Add to Cart  
 
Decanoyl-L-carnitine-d3 HCl (N-methyl-d3)
99%D
0.01 g $810     Add to Cart  
0.05 g $1,795     Add to Cart  
 
cis-4-Decenoic acid
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2-Decenoyl carnitine
5 mg $850     Add to Cart  
 
cis-4-Decenoyl carnitine
5 mg $900     Add to Cart  
 
2-Decenoyl N-(methyl-d3)-carnitine
98%D
5 mg $1,500     Add to Cart  
 
cis-4-Decenoyl N-(methyl-d3)-carnitine
98%D
5 mg $1,500     Add to Cart  
 
Desmosine-d4 inner salt
d0 = 0.02%, d1 = 0.25%, d2 = 5.19%, d3 = 26.11%, d4 = 65.55%, d5
1 mg $725     Add to Cart  
 
Ethyl trans-4-decenoate
5 g $990     Add to Cart  
 
Furan-2,5-dicarboxylic Acid
5 mg $1,200     Add to Cart  
 
2-Furoyl glycine
5 mg $790     Add to Cart  
 
Glutaroyl N-(trimethyl-d9)-carnitine
98%D
5 mg $690     Add to Cart  
 
Glutarylcarnitine
5 mg $1,400     Add to Cart  
 
Hexadecanoyl-L-carnitine-d3 HCl (N-methyl-d3)
98%D
0.01 g $780     Add to Cart  
0.05 g $1,695     Add to Cart  
 
Hexanoyl carnitine
5 mg $850     Add to Cart  
 
Hexanoyl glycine
5 mg $990     Add to Cart  
 
Hexanoyl-L-carnitine-d3 HCl (N-methyl-d3)
99%D
0.01 g $810     Add to Cart  
0.05 g $1,795     Add to Cart  
 
Homovanillic Acid
100 mg $150     Add to Cart  
1 g $450     Add to Cart  
 
2-Hydroxyadipic Acid
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4-Hydroxycyclohexylacetic acid
5 mg $1,300     Add to Cart  
 
3-Hydroxydodecanedioic Acid
5 mg $990     Add to Cart  
25 mg $2,490     Add to Cart  
 
3-Hydroxydodecanoic acid
25 mg $490     Add to Cart  
 
3-Hydroxyglutaric Acid
5 mg $890     Add to Cart  
 
5-Hydroxyhexanoic Acid
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3-Hydroxyisobutyric Acid
5 mg $190     Add to Cart  
25 mg $390     Add to Cart  
 
3-Hydroxyisovaleric Acid
5 mg $590     Add to Cart  
50 mg $990     Add to Cart  
 
Hydroxyisovaleroyl Carnitine
5 mg $1,200     Add to Cart  
 
Hydroxyisovaleroyl N-(Trimethyl-d9)-carnitine
98%D
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3-(3-Hydroxyphenyl)-3-hydroxypropionic Acid
100 mg $490     Add to Cart  
 
4-Hydroxyphenylacetyl glycine
5 mg $125     Add to Cart  
 
2-Hydroxyphytanic Acid
5 mg $490     Add to Cart  
 
3-Hydroxypropionic Acid (30% in Water)
5 mg $790     Add to Cart  
 
2-Hydroxysebacic Acid
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3-Hydroxysebacic Acid
5 mg $1,500     Add to Cart  
 
3-Hydroxysuberic Acid
5 mg $490     Add to Cart  
25 mg $1,490     Add to Cart  
 
3-Hydroxytetradecanedioic acid
1 mg $390     Add to Cart  
5 mg $1,250     Add to Cart  
 
Isobutyroyl glycine
5 mg $790     Add to Cart  
 
Isobutyroyl N-(methyl-d3)-Carnitine
98%D
5 mg $1,800     Add to Cart  
 
Isobutyryl Carnitine
5 mg $950     Add to Cart  
 
Isovaleric Acid
1 g $550     Add to Cart  
 
Isovaleroyl Carnitine
1 mg $290     Add to Cart  
5 mg $960     Add to Cart  
 
Isovaleroyl N-(methyl-d3)-Carnitine
98%D
5 mg $1,600     Add to Cart  
 
N-Isovaleroylglycine
5 mg $250     Add to Cart  
 
(-)-Menthol-1,2,6,6-d4
98% D
25 mg $890     Add to Cart  
 
Metacryloyl glycine
5 mg $790     Add to Cart  
 
2-Methylacetoacetic acid
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2-Methylbutyroyl Carnitine
5 mg $960     Add to Cart  
 
2-Methylbutyroyl glycine
5 mg $1,500     Add to Cart  
 
2-Methylbutyroyl N-(methyl-d3)-carnitine
5 mg $1,800     Add to Cart  
 
3-Methylcrotonyl Glycine
5 mg $790     Add to Cart  
 
N-(3-Methylcrotonyl)glycine-2,2-d2
98%D
10 mg $590     Add to Cart  
 
2-Methylglutaconic acid
5 mg $1,200     Add to Cart  
 
2-Methylmalonoyl carnitine
5 mg $960     Add to Cart  
 
2-Methylmalonoyl N-(methyl-d3)-carnitine
98%D
5 mg $2,200     Add to Cart  
 
2-Methylvaleroyl glycine
5 mg $790     Add to Cart  
 
Myristoyl Carnitine
5 mg $960     Add to Cart  
 
Myristoyl-L-Carnitine-d3 HCl (N-methyl-d3)
99%D
0.01 g $815     Add to Cart  
0.05 g $1,815     Add to Cart  
 
DL-Octanoyl carnitine chloride
1 mg $1,400     Add to Cart  
5 mg $2,500     Add to Cart  
 
Octanoyl-L-carnitine-d3 HCl (N-methyl-d3)
98%D
5 mg $750     Add to Cart  
 
2-Octene-1,8-dioic Acid
5 mg $1,300     Add to Cart  
 
2-Oxoadipic Acid
5 mg $1,350     Add to Cart  
 
3-Oxopentanoic Acid
5 mg $950     Add to Cart  
 
2-Oxophytanic Acid
1 mg Request Quote  
 
3-Hydroxy-3-phenylpropionic acid
5 mg $790     Add to Cart  
 
3-Phenylpropionyl glycine
10 mg $380     Add to Cart  
 
Propionyl-L-carnitine-d3 HCl (N-methyl-d3)
99%D
0.01 g $780     Add to Cart  
0.05 g $1,695     Add to Cart  
 
Stearoyl-L-carnitine-d3 HCl (N-methyl-d3)
99%D
0.01 g $810     Add to Cart  
0.05 g $1,795     Add to Cart  
 
cis-5-Tetradecenoic Acid
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Tiglyl glycine
5 mg $950     Add to Cart  
 
Valeroyl glycine
5 mg $1,500     Add to Cart  

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